- published: 03 Aug 2010
- views: 41988
- author: Farah Asad
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This health education video explains what phenylketonuria is. It discusses its causes, symptoms, diagnosis, and treatment.
My name is Kevin Alexander, I'm 33 years old, and I have PKU, also known as Phenylketonuria. This is my story. (If you'd like to connect with me I have a PKU...
- published: 23 Nov 2011
- views: 31738
- author: creativecontrolfilms
PK-Who is a documentary about individuals living with (PKU) Phenylketonuria and their challenges. This documentary seeks to raise awareness about the dire co...
- published: 29 May 2010
- views: 13610
- author: Tucker Parris
Jedyny profil Molesty Ewenement: http://www.facebook.com/molestaofficial Więcej teledysków możecie znaleźć tutaj http://www.youtube.com/molestatv.
- published: 10 Mar 2013
- views: 5622
- author: MC5gramTV
Phenylketonuria (PKU) is caused by a mutation to a recessive gene on chromosome 12. This gene would normally convert the amino acid phenylalanine into the am...
- published: 10 Feb 2013
- views: 5449
- author: Stephanie Castle
This video tells the story of Lauren, a successful student and athlete who began taking Kuvan, and decided to do a school science fair project on her experie...
- published: 08 Aug 2008
- views: 25092
- author: ProjectKuvan
A short documentary on the genetic, metabolic disorder Phenylketonuria (PKU.) Gives a brief overview.
- published: 18 Jan 2008
- views: 44162
- author: clarelj17
Artist: Toony; Title: PKU; File: Video; Subscribe our ProstoTV channel Subskrybuj nasz kanal ProstoTV.
- published: 05 Jan 2009
- views: 479259
- author: ProstoTV
This footage shows a newborn baby's heel being pricked to collect drops of blood to screen for PKU and other rare, genetic disorders. PKU is recognized as th...
- published: 26 Oct 2012
- views: 2891
- author: canpku
RAISING A CHILD WHO HAS PKU: How two moms, with sons born 20 years apart, learned to cope.
- published: 16 Jul 2012
- views: 1135
- author: SaveBabiesFoundation
Mit diesem Film soll Müttern von neuen PKU-Patienten die Angst genommen werden, er soll persönliche Erfahrungen zeigen und allgemeine Informationen liefern!
https://www.facebook.com/pages/Tobi-LA/1498245937112807
- published: 08 Mar 2015
- views: 26
The pku, alternatively spelled pzuk, is an Armenian musical instrument, similar to a clarinet. It has been called the national instrument of Armenia. The pku is a single-reed aerophone with seven holes and a one octave range with the open cone of a bull horn at one end.
The pku is an ancient musical instrument, originating from the 5th century. Today a few types of the instrument are known in Armenia, which are the timbre supplementation of each other and have rather high diapason. The instrument consists of a pipe and a reed. A horn prepared from a cow’s horn is fixed to the lower part of the pipe. The reed is prepared from ebonite and a cane mouthpiece. The mouthpiece can produce shrill or soft sounds. The instrument can be prepared in different keys - in C, A, D, F, B, etc.
This page contains text from Wikipedia, the Free Encyclopedia -
http://en.wikipedia.org/wiki/Pku
This article is licensed under the Creative Commons Attribution-ShareAlike 3.0 Unported License, which means that you can copy and modify it as long as the entire work (including additions) remains under this license.
This article is licensed under the Creative Commons Attribution-ShareAlike 3.0 Unported License, which means that you can copy and modify it as long as the entire work (including additions) remains under this license.
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3:01Phenylketonuria or PKU
Phenylketonuria or PKUPhenylketonuria or PKU
This health education video explains what phenylketonuria is. It discusses its causes, symptoms, diagnosis, and treatment. -
11:28My PKU Life
My PKU LifeMy PKU Life
My name is Kevin Alexander, I'm 33 years old, and I have PKU, also known as Phenylketonuria. This is my story. (If you'd like to connect with me I have a PKU... -
10:37PK-Who: PKU Documentary
PK-Who: PKU DocumentaryPK-Who: PKU Documentary
PK-Who is a documentary about individuals living with (PKU) Phenylketonuria and their challenges. This documentary seeks to raise awareness about the dire co... -
3:27Molesta - PKU - Patrz Komu Ufasz (SKANDAL) HQ
Molesta - PKU - Patrz Komu Ufasz (SKANDAL) HQMolesta - PKU - Patrz Komu Ufasz (SKANDAL) HQ
Jedyny profil Molesty Ewenement: http://www.facebook.com/molestaofficial Więcej teledysków możecie znaleźć tutaj http://www.youtube.com/molestatv. -
1:23A.1.5 Explain the causes and consequences of phenylketonuria (PKU)
A.1.5 Explain the causes and consequences of phenylketonuria (PKU)A.1.5 Explain the causes and consequences of phenylketonuria (PKU)
Phenylketonuria (PKU) is caused by a mutation to a recessive gene on chromosome 12. This gene would normally convert the amino acid phenylalanine into the am... -
7:58Lauren's PKU Story
Lauren's PKU StoryLauren's PKU Story
This video tells the story of Lauren, a successful student and athlete who began taking Kuvan, and decided to do a school science fair project on her experie... -
6:02PKU
PKUPKU
A short documentary on the genetic, metabolic disorder Phenylketonuria (PKU.) Gives a brief overview. -
3:23Toony - PKU
Toony - PKUToony - PKU
Artist: Toony; Title: PKU; File: Video; Subscribe our ProstoTV channel Subskrybuj nasz kanal ProstoTV. -
1:20Newborn screening for PKU
Newborn screening for PKUNewborn screening for PKU
This footage shows a newborn baby's heel being pricked to collect drops of blood to screen for PKU and other rare, genetic disorders. PKU is recognized as th... -
7:25Raising A Child With PKU - Newborn Screening Saves Babies
Raising A Child With PKU - Newborn Screening Saves BabiesRaising A Child With PKU - Newborn Screening Saves Babies
RAISING A CHILD WHO HAS PKU: How two moms, with sons born 20 years apart, learned to cope. -
3:27Extreme Machines Vehicle ZIL PKU 1 Amphibian
Extreme Machines Vehicle ZIL PKU 1 AmphibianExtreme Machines Vehicle ZIL PKU 1 Amphibian
-
6:31DOKUMENTATION: Wir haben PKU, nicht die PKU hat uns!
DOKUMENTATION: Wir haben PKU, nicht die PKU hat uns!DOKUMENTATION: Wir haben PKU, nicht die PKU hat uns!
Mit diesem Film soll Müttern von neuen PKU-Patienten die Angst genommen werden, er soll persönliche Erfahrungen zeigen und allgemeine Informationen liefern! https://www.facebook.com/pages/Tobi-LA/1498245937112807 -
10:52《我是演說家》六歲李欣睿PKU患者
《我是演說家》六歲李欣睿PKU患者《我是演說家》六歲李欣睿PKU患者
苯酮尿症(Phenylketonuria)，簡稱 PKU。 1934年挪威的 Dr.Folling在家族性智障病患的尿液中發現有特殊陳腐味道，後來才知道該物質是苯丙酮酸(phenylpyruvic acid)，這和尿中有糖的糖尿病是無關的，在 1937年正式命名為苯酮尿症(phenylketonuria；PKU)。 致病機轉：異型苯酮尿症(BH4缺乏症)是一種體染色體隱性遺傳的胺基酸代謝異常疾病。苯丙胺酸(phenylalanine)為人體的必須胺基酸，其主要的代謝反應為苯丙胺酸(PHE)經苯丙胺酸羥化脢(PAH)、BH4輔脢等物質作用而羥化成酪胺酸 (Tyrosine)。異型苯酮尿症是因為 PAH羥化反應所需之 BH4輔脢 (Tetrahydrobiopterin) 的代謝過程發生機障，無法產生足夠的 BH4供給 PAH使用，導致苯丙胺酸無法順利轉換成酪胺酸，而在體內大量堆積，進而產生許多有毒的代謝產物。在正常的情況下， BH4是由 GTP經過一連串的反應，包含 GTP-Cyclohydrolase和 6-Pyruvoyltetrahydropterin Synthase(6PTS)等酵素的作用而合成的。參與苯丙胺酸羥化水解反應後必須經由 Dihydropteridine Reductase DHPR還原為 BH4形成一個網路循環應用。 BH4不但參與苯丙胺酸羥化成酪胺酸，也參與酪胺酸羥化成 Dopa和色胺酸(Tryptophan) 羥化成 5-OH Tryptophan的 -
3:24Living With PKU
Living With PKULiving With PKU
- Alboka
- Arghul
- Armenia
- Aulochrome
- Bass clarinet
- Chalumeau
- Clarinet
- Dili tuiduk
- Diplica
- Double clarinet
- E-flat Clarinet
- Heckel-clarina
- Heckelphone-clarinet
- Horn (instrument)
- JSTOR
- Launeddas
- Mijwiz
- National instrument
- Octavin
- Pibgorn (instrument)
- Pipe (material)
- Pku
- PKU (disambiguation)
- Pungi
- Saxophone
- Sipsi
- Stock-and-horn
- Timbre
- Tárogató
- Xaphoon
- Zhaleika
-
Phenylketonuria or PKU
This health education video explains what phenylketonuria is. It discusses its causes, symptoms, diagnosis, and treatment. -
My PKU Life
My name is Kevin Alexander, I'm 33 years old, and I have PKU, also known as Phenylketonuria. This is my story. (If you'd like to connect with me I have a PKU... -
PK-Who: PKU Documentary
PK-Who is a documentary about individuals living with (PKU) Phenylketonuria and their challenges. This documentary seeks to raise awareness about the dire co... -
Molesta - PKU - Patrz Komu Ufasz (SKANDAL) HQ
Jedyny profil Molesty Ewenement: http://www.facebook.com/molestaofficial Więcej teledysków możecie znaleźć tutaj http://www.youtube.com/molestatv. -
A.1.5 Explain the causes and consequences of phenylketonuria (PKU)
Phenylketonuria (PKU) is caused by a mutation to a recessive gene on chromosome 12. This gene would normally convert the amino acid phenylalanine into the am... -
Lauren's PKU Story
This video tells the story of Lauren, a successful student and athlete who began taking Kuvan, and decided to do a school science fair project on her experie... -
PKU
A short documentary on the genetic, metabolic disorder Phenylketonuria (PKU.) Gives a brief overview. -
Toony - PKU
Artist: Toony; Title: PKU; File: Video; Subscribe our ProstoTV channel Subskrybuj nasz kanal ProstoTV. -
Newborn screening for PKU
This footage shows a newborn baby's heel being pricked to collect drops of blood to screen for PKU and other rare, genetic disorders. PKU is recognized as th... -
Raising A Child With PKU - Newborn Screening Saves Babies
RAISING A CHILD WHO HAS PKU: How two moms, with sons born 20 years apart, learned to cope. -
Extreme Machines Vehicle ZIL PKU 1 Amphibian
-
DOKUMENTATION: Wir haben PKU, nicht die PKU hat uns!
Mit diesem Film soll Müttern von neuen PKU-Patienten die Angst genommen werden, er soll persönliche Erfahrungen zeigen und allgemeine Informationen liefern! https://www.facebook.com/pages/Tobi-LA/1498245937112807 -
《我是演說家》六歲李欣睿PKU患者
苯酮尿症(Phenylketonuria)，簡稱 PKU。 1934年挪威的 Dr.Folling在家族性智障病患的尿液中發現有特殊陳腐味道，後來才知道該物質是苯丙酮酸(phenylpyruvic acid)，這和尿中有糖的糖尿病是無關的，在 1937年正式命名為苯酮尿症(phenylketonuria；PKU)。 致病機轉：異型苯酮尿症(BH4缺乏症)是一種體染色體隱性遺傳的胺基酸代謝異常疾病。苯丙胺酸(phenylalanine)為人體的必須胺基酸，其主要的代謝反應為苯丙胺酸(PHE)經苯丙胺酸羥化脢(PAH)、BH4輔脢等物質作用而羥化成酪胺酸 (Tyrosine)。異型苯酮尿症是因為 PAH羥化反應所需之 BH4輔脢 (Tetrahydrobiopterin) 的代謝過程發生機障，無法產生足夠的 BH4供給 PAH使用，導致苯丙胺酸無法順利轉換成酪胺酸，而在體內大量堆積，進而產 -
Living With PKU
-
Molesta - P.K.U (Patrz Komu Ufasz)
-
PKU Child Symptoms
-
PKU Heel Prick
Check out our blog: http://mylittlestsweetpea.blogspot.com/ Our one year old daughter has classic PKU (phenylketonuria) and has to have weekly blood draws.... -
The faces of PKU .mov
Phenylketonuria (commonly known as PKU) is a rare genetic disorder affecting protein metabolism. Individuals with PKU cannot process the amino acid phenylala... -
Mothers, Babies and PKU
Almost 50 years after newborns in the United States started being tested for PKU, a genetic disorder that requires a special diet, no one knows how many of t... -
ARTHUR PONCH pku ARMAN drums JEYMS dhol
ARTHUR PONCH pku ARMAN drums JEYMS dhol -
Gev(pku),Lyov(dhol),Aram(acomp.),Varuj(drums),Arman(solo)
-
PKU, la enfermedad metabólica más común
La fenilcetonuria, también conocida como PKU (del inglés phenylketonuria = PKU), es un error congénito del metabolismo causado por la carencia de la enzima f... -
My PKU Life (4 Minute Version)
My name is Kevin Alexander, I'm 31 years old, and I have PKU, also known as Phenylketonuria. This is my story. Help us caption & translate this video! http:/...
Phenylketonuria or PKU
- Order: Reorder
- Duration: 3:01
- Updated: 30 Aug 2014
- views: 41988
- author: Farah Asad
This health education video explains what phenylketonuria is. It discusses its causes, symptoms, diagnosis, and treatment....
This health education video explains what phenylketonuria is. It discusses its causes, symptoms, diagnosis, and treatment.
wn.com/Phenylketonuria Or Pku
This health education video explains what phenylketonuria is. It discusses its causes, symptoms, diagnosis, and treatment.
- published: 03 Aug 2010
- views: 41988
- author: Farah Asad
My PKU Life
- Order: Reorder
- Duration: 11:28
- Updated: 06 Sep 2014
- views: 31738
- author: creativecontrolfilms
My name is Kevin Alexander, I'm 33 years old, and I have PKU, also known as Phenylketonuria. This is my story. (If you'd like to connect with me I have a PKU......
My name is Kevin Alexander, I'm 33 years old, and I have PKU, also known as Phenylketonuria. This is my story. (If you'd like to connect with me I have a PKU...
wn.com/My Pku Life
My name is Kevin Alexander, I'm 33 years old, and I have PKU, also known as Phenylketonuria. This is my story. (If you'd like to connect with me I have a PKU...
- published: 23 Nov 2011
- views: 31738
- author: creativecontrolfilms
PK-Who: PKU Documentary
- Order: Reorder
- Duration: 10:37
- Updated: 26 Aug 2014
- views: 13610
- author: Tucker Parris
PK-Who is a documentary about individuals living with (PKU) Phenylketonuria and their challenges. This documentary seeks to raise awareness about the dire co......
PK-Who is a documentary about individuals living with (PKU) Phenylketonuria and their challenges. This documentary seeks to raise awareness about the dire co...
wn.com/Pk Who Pku Documentary
PK-Who is a documentary about individuals living with (PKU) Phenylketonuria and their challenges. This documentary seeks to raise awareness about the dire co...
- published: 29 May 2010
- views: 13610
- author: Tucker Parris
Molesta - PKU - Patrz Komu Ufasz (SKANDAL) HQ
Jedyny profil Molesty Ewenement: http://www.facebook.com/molestaofficial Więcej teledysków możecie znaleźć tutaj http://www.youtube.com/molestatv....
Jedyny profil Molesty Ewenement: http://www.facebook.com/molestaofficial Więcej teledysków możecie znaleźć tutaj http://www.youtube.com/molestatv.
wn.com/Molesta Pku Patrz Komu Ufasz (Skandal) Hq
Jedyny profil Molesty Ewenement: http://www.facebook.com/molestaofficial Więcej teledysków możecie znaleźć tutaj http://www.youtube.com/molestatv.
- published: 10 Mar 2013
- views: 5622
- author: MC5gramTV
A.1.5 Explain the causes and consequences of phenylketonuria (PKU)
- Order: Reorder
- Duration: 1:23
- Updated: 02 Apr 2014
- views: 5449
- author: Stephanie Castle
Phenylketonuria (PKU) is caused by a mutation to a recessive gene on chromosome 12. This gene would normally convert the amino acid phenylalanine into the am......
Phenylketonuria (PKU) is caused by a mutation to a recessive gene on chromosome 12. This gene would normally convert the amino acid phenylalanine into the am...
wn.com/A.1.5 Explain The Causes And Consequences Of Phenylketonuria (Pku)
Phenylketonuria (PKU) is caused by a mutation to a recessive gene on chromosome 12. This gene would normally convert the amino acid phenylalanine into the am...
- published: 10 Feb 2013
- views: 5449
- author: Stephanie Castle
Lauren's PKU Story
- Order: Reorder
- Duration: 7:58
- Updated: 23 Aug 2014
- views: 25092
- author: ProjectKuvan
This video tells the story of Lauren, a successful student and athlete who began taking Kuvan, and decided to do a school science fair project on her experie......
This video tells the story of Lauren, a successful student and athlete who began taking Kuvan, and decided to do a school science fair project on her experie...
wn.com/Lauren's Pku Story
This video tells the story of Lauren, a successful student and athlete who began taking Kuvan, and decided to do a school science fair project on her experie...
- published: 08 Aug 2008
- views: 25092
- author: ProjectKuvan
PKU
A short documentary on the genetic, metabolic disorder Phenylketonuria (PKU.) Gives a brief overview....
A short documentary on the genetic, metabolic disorder Phenylketonuria (PKU.) Gives a brief overview.
wn.com/Pku
A short documentary on the genetic, metabolic disorder Phenylketonuria (PKU.) Gives a brief overview.
- published: 18 Jan 2008
- views: 44162
- author: clarelj17
Toony - PKU
Artist: Toony; Title: PKU; File: Video; Subscribe our ProstoTV channel Subskrybuj nasz kanal ProstoTV....
Artist: Toony; Title: PKU; File: Video; Subscribe our ProstoTV channel Subskrybuj nasz kanal ProstoTV.
wn.com/Toony Pku
Artist: Toony; Title: PKU; File: Video; Subscribe our ProstoTV channel Subskrybuj nasz kanal ProstoTV.
- published: 05 Jan 2009
- views: 479259
- author: ProstoTV
Newborn screening for PKU
This footage shows a newborn baby's heel being pricked to collect drops of blood to screen for PKU and other rare, genetic disorders. PKU is recognized as th......
This footage shows a newborn baby's heel being pricked to collect drops of blood to screen for PKU and other rare, genetic disorders. PKU is recognized as th...
wn.com/Newborn Screening For Pku
This footage shows a newborn baby's heel being pricked to collect drops of blood to screen for PKU and other rare, genetic disorders. PKU is recognized as th...
- published: 26 Oct 2012
- views: 2891
- author: canpku
Raising A Child With PKU - Newborn Screening Saves Babies
- Order: Reorder
- Duration: 7:25
- Updated: 11 Dec 2012
- views: 1135
- author: SaveBabiesFoundation
RAISING A CHILD WHO HAS PKU: How two moms, with sons born 20 years apart, learned to cope....
RAISING A CHILD WHO HAS PKU: How two moms, with sons born 20 years apart, learned to cope.
wn.com/Raising A Child With Pku Newborn Screening Saves Babies
RAISING A CHILD WHO HAS PKU: How two moms, with sons born 20 years apart, learned to cope.
- published: 16 Jul 2012
- views: 1135
- author: SaveBabiesFoundation
Extreme Machines Vehicle ZIL PKU 1 Amphibian
- Order: Reorder
- Duration: 3:27
- Updated: 27 Aug 2015
- views: 985
...
wn.com/Extreme Machines Vehicle Zil Pku 1 Amphibian
- published: 27 Aug 2015
- views: 985
DOKUMENTATION: Wir haben PKU, nicht die PKU hat uns!
- Order: Reorder
- Duration: 6:31
- Updated: 08 Mar 2015
- views: 26
Mit diesem Film soll Müttern von neuen PKU-Patienten die Angst genommen werden, er soll persönliche Erfahrungen zeigen und allgemeine Informationen liefern!
htt...
Mit diesem Film soll Müttern von neuen PKU-Patienten die Angst genommen werden, er soll persönliche Erfahrungen zeigen und allgemeine Informationen liefern!
https://www.facebook.com/pages/Tobi-LA/1498245937112807
wn.com/Dokumentation Wir Haben Pku, Nicht Die Pku Hat Uns
Mit diesem Film soll Müttern von neuen PKU-Patienten die Angst genommen werden, er soll persönliche Erfahrungen zeigen und allgemeine Informationen liefern!
https://www.facebook.com/pages/Tobi-LA/1498245937112807
- published: 08 Mar 2015
- views: 26
《我是演說家》六歲李欣睿PKU患者
- Order: Reorder
- Duration: 10:52
- Updated: 09 Nov 2014
- views: 38
苯酮尿症(Phenylketonuria)，簡稱 PKU。
1934年挪威的 Dr.Folling在家族性智障病患的尿液中發現有特殊陳腐味道，後來才知道該物質是苯丙酮酸(phenylpyruvic acid)，這和尿中有糖的糖尿病是無關的，在 1937年正式命名為苯酮尿症(phenylketonuria；PKU)。
...
苯酮尿症(Phenylketonuria)，簡稱 PKU。
1934年挪威的 Dr.Folling在家族性智障病患的尿液中發現有特殊陳腐味道，後來才知道該物質是苯丙酮酸(phenylpyruvic acid)，這和尿中有糖的糖尿病是無關的，在 1937年正式命名為苯酮尿症(phenylketonuria；PKU)。
致病機轉：異型苯酮尿症(BH4缺乏症)是一種體染色體隱性遺傳的胺基酸代謝異常疾病。苯丙胺酸(phenylalanine)為人體的必須胺基酸，其主要的代謝反應為苯丙胺酸(PHE)經苯丙胺酸羥化脢(PAH)、BH4輔脢等物質作用而羥化成酪胺酸 (Tyrosine)。異型苯酮尿症是因為 PAH羥化反應所需之 BH4輔脢 (Tetrahydrobiopterin) 的代謝過程發生機障，無法產生足夠的 BH4供給 PAH使用，導致苯丙胺酸無法順利轉換成酪胺酸，而在體內大量堆積，進而產生許多有毒的代謝產物。在正常的情況下， BH4是由 GTP經過一連串的反應，包含 GTP-Cyclohydrolase和 6-Pyruvoyltetrahydropterin Synthase(6PTS)等酵素的作用而合成的。參與苯丙胺酸羥化水解反應後必須經由 Dihydropteridine Reductase DHPR還原為 BH4形成一個網路循環應用。 BH4不但參與苯丙胺酸羥化成酪胺酸，也參與酪胺酸羥化成 Dopa和色胺酸(Tryptophan) 羥化成 5-OH Tryptophan的反應。其中 Dopa和 5-OH Tryptophan為中樞神經傳導物質 Dopamine和 Serotonin的先驅物質。因此若缺乏 BH4輔脢，則三項反應均會受阻，此時不但會出現高苯丙胺酸之症狀，同時也因中樞神經傳導物質的缺乏，而出現許多神經學的症狀。目前已知有下列五種不同酵素的缺乏會造成苯酮尿症代謝機轉：
1.苯丙胺酸羥化酵素 ( PAH )
2.鳥嘌呤核甘三磷酸環化水解酵素 ( GTPCHI )
3.丙二酮四氫喋呤合成酵素 ( PTPS )
4.雙氫喋啶還原酵素 ( DHPR )
5.喋呤甲醇胺脫水酵素 ( PCD )
wn.com/《我是演說家》六歲李欣睿Pku患者
苯酮尿症(Phenylketonuria)，簡稱 PKU。
1934年挪威的 Dr.Folling在家族性智障病患的尿液中發現有特殊陳腐味道，後來才知道該物質是苯丙酮酸(phenylpyruvic acid)，這和尿中有糖的糖尿病是無關的，在 1937年正式命名為苯酮尿症(phenylketonuria；PKU)。
致病機轉：異型苯酮尿症(BH4缺乏症)是一種體染色體隱性遺傳的胺基酸代謝異常疾病。苯丙胺酸(phenylalanine)為人體的必須胺基酸，其主要的代謝反應為苯丙胺酸(PHE)經苯丙胺酸羥化脢(PAH)、BH4輔脢等物質作用而羥化成酪胺酸 (Tyrosine)。異型苯酮尿症是因為 PAH羥化反應所需之 BH4輔脢 (Tetrahydrobiopterin) 的代謝過程發生機障，無法產生足夠的 BH4供給 PAH使用，導致苯丙胺酸無法順利轉換成酪胺酸，而在體內大量堆積，進而產生許多有毒的代謝產物。在正常的情況下， BH4是由 GTP經過一連串的反應，包含 GTP-Cyclohydrolase和 6-Pyruvoyltetrahydropterin Synthase(6PTS)等酵素的作用而合成的。參與苯丙胺酸羥化水解反應後必須經由 Dihydropteridine Reductase DHPR還原為 BH4形成一個網路循環應用。 BH4不但參與苯丙胺酸羥化成酪胺酸，也參與酪胺酸羥化成 Dopa和色胺酸(Tryptophan) 羥化成 5-OH Tryptophan的反應。其中 Dopa和 5-OH Tryptophan為中樞神經傳導物質 Dopamine和 Serotonin的先驅物質。因此若缺乏 BH4輔脢，則三項反應均會受阻，此時不但會出現高苯丙胺酸之症狀，同時也因中樞神經傳導物質的缺乏，而出現許多神經學的症狀。目前已知有下列五種不同酵素的缺乏會造成苯酮尿症代謝機轉：
1.苯丙胺酸羥化酵素 ( PAH )
2.鳥嘌呤核甘三磷酸環化水解酵素 ( GTPCHI )
3.丙二酮四氫喋呤合成酵素 ( PTPS )
4.雙氫喋啶還原酵素 ( DHPR )
5.喋呤甲醇胺脫水酵素 ( PCD )
- published: 09 Nov 2014
- views: 38
Molesta - P.K.U (Patrz Komu Ufasz)
- Order: Reorder
- Duration: 4:40
- Updated: 13 Aug 2014
- views: 22646
- author: TVMolestaEwenement
...
wn.com/Molesta P.K.U (Patrz Komu Ufasz)
- published: 31 Aug 2012
- views: 22646
- author: TVMolestaEwenement
PKU Child Symptoms
- Order: Reorder
- Duration: 5:27
- Updated: 10 Aug 2014
- views: 2677
- author: psteezy626
...
wn.com/Pku Child Symptoms
- published: 06 Feb 2013
- views: 2677
- author: psteezy626
PKU Heel Prick
- Order: Reorder
- Duration: 7:22
- Updated: 30 Aug 2014
- views: 22224
- author: Littlest Sweet Pea.com
Check out our blog: http://mylittlestsweetpea.blogspot.com/ Our one year old daughter has classic PKU (phenylketonuria) and has to have weekly blood draws.......
Check out our blog: http://mylittlestsweetpea.blogspot.com/ Our one year old daughter has classic PKU (phenylketonuria) and has to have weekly blood draws....
wn.com/Pku Heel Prick
Check out our blog: http://mylittlestsweetpea.blogspot.com/ Our one year old daughter has classic PKU (phenylketonuria) and has to have weekly blood draws....
- published: 14 Oct 2010
- views: 22224
- author: Littlest Sweet Pea.com
The faces of PKU .mov
- Order: Reorder
- Duration: 1:36
- Updated: 30 Jul 2014
- views: 4415
- author: Joanne Hunt
Phenylketonuria (commonly known as PKU) is a rare genetic disorder affecting protein metabolism. Individuals with PKU cannot process the amino acid phenylala......
Phenylketonuria (commonly known as PKU) is a rare genetic disorder affecting protein metabolism. Individuals with PKU cannot process the amino acid phenylala...
wn.com/The Faces Of Pku .Mov
Phenylketonuria (commonly known as PKU) is a rare genetic disorder affecting protein metabolism. Individuals with PKU cannot process the amino acid phenylala...
- published: 19 May 2012
- views: 4415
- author: Joanne Hunt
Mothers, Babies and PKU
- Order: Reorder
- Duration: 2:08
- Updated: 30 Aug 2014
- views: 3226
- author: swedishseattle
Almost 50 years after newborns in the United States started being tested for PKU, a genetic disorder that requires a special diet, no one knows how many of t......
Almost 50 years after newborns in the United States started being tested for PKU, a genetic disorder that requires a special diet, no one knows how many of t...
wn.com/Mothers, Babies And Pku
Almost 50 years after newborns in the United States started being tested for PKU, a genetic disorder that requires a special diet, no one knows how many of t...
- published: 01 Aug 2012
- views: 3226
- author: swedishseattle
ARTHUR PONCH pku ARMAN drums JEYMS dhol
- Order: Reorder
- Duration: 5:15
- Updated: 29 Sep 2014
- views: 368
ARTHUR PONCH pku ARMAN drums JEYMS dhol...
ARTHUR PONCH pku ARMAN drums JEYMS dhol
wn.com/Arthur Ponch Pku Arman Drums Jeyms Dhol
ARTHUR PONCH pku ARMAN drums JEYMS dhol
- published: 29 Sep 2014
- views: 368
Gev(pku),Lyov(dhol),Aram(acomp.),Varuj(drums),Arman(solo)
- Order: Reorder
- Duration: 9:00
- Updated: 11 Aug 2014
- views: 493
- author: Karen Harutyunyan
...
wn.com/Gev(Pku),Lyov(Dhol),Aram(Acomp.),Varuj(Drums),Arman(Solo)
- published: 09 Aug 2014
- views: 493
- author: Karen Harutyunyan
PKU, la enfermedad metabólica más común
- Order: Reorder
- Duration: 2:59
- Updated: 21 Aug 2014
- views: 1536
- author: Hospital Sant Joan de Déu
La fenilcetonuria, también conocida como PKU (del inglés phenylketonuria = PKU), es un error congénito del metabolismo causado por la carencia de la enzima f......
La fenilcetonuria, también conocida como PKU (del inglés phenylketonuria = PKU), es un error congénito del metabolismo causado por la carencia de la enzima f...
wn.com/Pku, La Enfermedad Metabólica Más Común
La fenilcetonuria, también conocida como PKU (del inglés phenylketonuria = PKU), es un error congénito del metabolismo causado por la carencia de la enzima f...
- published: 22 Jul 2013
- views: 1536
- author: Hospital Sant Joan de Déu
My PKU Life (4 Minute Version)
- Order: Reorder
- Duration: 4:24
- Updated: 27 Aug 2014
- views: 3807
- author: creativecontrolfilms
My name is Kevin Alexander, I'm 31 years old, and I have PKU, also known as Phenylketonuria. This is my story. Help us caption & translate this video! http:/......
My name is Kevin Alexander, I'm 31 years old, and I have PKU, also known as Phenylketonuria. This is my story. Help us caption & translate this video! http:/...
wn.com/My Pku Life (4 Minute Version)
My name is Kevin Alexander, I'm 31 years old, and I have PKU, also known as Phenylketonuria. This is my story. Help us caption & translate this video! http:/...
- published: 23 Jun 2012
- views: 3807
- author: creativecontrolfilms
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3:01
Phenylketonuria or PKU
This health education video explains what phenylketonuria is. It discusses its causes, sym...
published: 03 Aug 2010
author: Farah Asad
Phenylketonuria or PKU
Phenylketonuria or PKU
- published: 03 Aug 2010
- views: 41988
-
author:
Farah Asad
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11:28
My PKU Life
My name is Kevin Alexander, I'm 33 years old, and I have PKU, also known as Phenylketonuri...
published: 23 Nov 2011
author: creativecontrolfilms
My PKU Life
My PKU Life
- published: 23 Nov 2011
- views: 31738
-
author:
creativecontrolfilms
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10:37
PK-Who: PKU Documentary
PK-Who is a documentary about individuals living with (PKU) Phenylketonuria and their chal...
published: 29 May 2010
author: Tucker Parris
PK-Who: PKU Documentary
PK-Who: PKU Documentary
- published: 29 May 2010
- views: 13610
-
author:
Tucker Parris
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3:27
Molesta - PKU - Patrz Komu Ufasz (SKANDAL) HQ
Jedyny profil Molesty Ewenement: http://www.facebook.com/molestaofficial Więcej teledysków...
published: 10 Mar 2013
author: MC5gramTV
Molesta - PKU - Patrz Komu Ufasz (SKANDAL) HQ
Molesta - PKU - Patrz Komu Ufasz (SKANDAL) HQ
- published: 10 Mar 2013
- views: 5622
-
author:
MC5gramTV
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1:23
A.1.5 Explain the causes and consequences of phenylketonuria (PKU)
Phenylketonuria (PKU) is caused by a mutation to a recessive gene on chromosome 12. This g...
published: 10 Feb 2013
author: Stephanie Castle
A.1.5 Explain the causes and consequences of phenylketonuria (PKU)
A.1.5 Explain the causes and consequences of phenylketonuria (PKU)
- published: 10 Feb 2013
- views: 5449
-
author:
Stephanie Castle
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7:58
Lauren's PKU Story
This video tells the story of Lauren, a successful student and athlete who began taking Ku...
published: 08 Aug 2008
author: ProjectKuvan
Lauren's PKU Story
Lauren's PKU Story
- published: 08 Aug 2008
- views: 25092
-
author:
ProjectKuvan
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6:02
PKU
A short documentary on the genetic, metabolic disorder Phenylketonuria (PKU.) Gives a brie...
published: 18 Jan 2008
author: clarelj17
PKU
PKU
- published: 18 Jan 2008
- views: 44162
-
author:
clarelj17
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3:23
Toony - PKU
Artist: Toony; Title: PKU; File: Video; Subscribe our ProstoTV channel Subskrybuj nasz kan...
published: 05 Jan 2009
author: ProstoTV
Toony - PKU
Toony - PKU
- published: 05 Jan 2009
- views: 479259
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author:
ProstoTV
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1:20
Newborn screening for PKU
This footage shows a newborn baby's heel being pricked to collect drops of blood to screen...
published: 26 Oct 2012
author: canpku
Newborn screening for PKU
Newborn screening for PKU
- published: 26 Oct 2012
- views: 2891
-
author:
canpku
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7:25
Raising A Child With PKU - Newborn Screening Saves Babies
RAISING A CHILD WHO HAS PKU: How two moms, with sons born 20 years apart, learned to cope....
published: 16 Jul 2012
author: SaveBabiesFoundation
Raising A Child With PKU - Newborn Screening Saves Babies
Raising A Child With PKU - Newborn Screening Saves Babies
- published: 16 Jul 2012
- views: 1135
-
author:
SaveBabiesFoundation
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3:27
Extreme Machines Vehicle ZIL PKU 1 Amphibian
...
published: 27 Aug 2015
Extreme Machines Vehicle ZIL PKU 1 Amphibian
Extreme Machines Vehicle ZIL PKU 1 Amphibian
- published: 27 Aug 2015
- views: 985
6:31
DOKUMENTATION: Wir haben PKU, nicht die PKU hat uns!
Mit diesem Film soll Müttern von neuen PKU-Patienten die Angst genommen werden, er soll pe...
published: 08 Mar 2015
DOKUMENTATION: Wir haben PKU, nicht die PKU hat uns!
DOKUMENTATION: Wir haben PKU, nicht die PKU hat uns!
- published: 08 Mar 2015
- views: 26
10:52
《我是演說家》六歲李欣睿PKU患者
苯酮尿症(Phenylketonuria)，簡稱 PKU。
1934年挪威的 Dr.Folling在家族性智障病患的尿液中發現有特殊陳腐味道，後來才知道該物質是苯丙酮酸(pheny...
published: 09 Nov 2014
《我是演說家》六歲李欣睿PKU患者
《我是演說家》六歲李欣睿PKU患者
- published: 09 Nov 2014
- views: 38
3:24
Living With PKU
...
published: 19 Jan 2012
author: TexanATO
Living With PKU
Living With PKU
- published: 19 Jan 2012
- views: 5940
-
author:
TexanATO
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Phenylketonuria or PKU...
My PKU Life...
PK-Who: PKU Documentary...
Molesta - PKU - Patrz Komu Ufasz (SKANDAL) HQ...
A.1.5 Explain the causes and consequences of pheny...
Lauren's PKU Story...
PKU...
Toony - PKU...
Newborn screening for PKU...
Raising A Child With PKU - Newborn Screening Saves...
Extreme Machines Vehicle ZIL PKU 1 Amphibian...
DOKUMENTATION: Wir haben PKU, nicht die PKU hat un...
《我是演說家》六歲李欣睿PKU患者...
Living With PKU...
Molesta - P.K.U (Patrz Komu Ufasz)...
PKU Child Symptoms...
PKU Heel Prick...
The faces of PKU .mov...
Mothers, Babies and PKU...
ARTHUR PONCH pku ARMAN drums JEYMS dhol...
Gev(pku),Lyov(dhol),Aram(acomp.),Varuj(drums),Arma...
PKU, la enfermedad metabólica más común...
My PKU Life (4 Minute Version)...
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photo: AP / Pat Sullivan
Teresa Laraba, Southwest Airlines Service Leader, Dies at 53
Edit Bloomberg 26 Dec 2015
Teresa Laraba, one of the U.S. airline industry’s highest-ranking female executives as Southwest Airlines Co.’s head of customer services, has died at the age of 53. Laraba rose to prominence at the Dallas-based carrier after starting as an entry-level customer service agent in 1984, the company said Saturday in a statement ... ....
photo: creative commons
Unprecedented Weather In United Kingdom, US Blamed on El Nino, Climate-Change Phenomenon
Edit WorldNews.com 28 Dec 2015
El Niño and climate change have provoked unprecedented weather phenomenon around the globe with flooding in the United Kingdom, an unusually warm winter in the Northeast United States and tornadoes and freakish snowstorms in the Southwest U.S., according to multiple media reports. According to a Monday Inquistr report, there were more than 200 flood alerts on Dec. 28 – including 20 life-threatening “severe warnings ... ....
photo: AP / Hani Mohammed
Bury My Dreams And Heart At Wounded Yemen
Edit WorldNews.com 28 Dec 2015
“Don’t bury me” cried 6-year Fareed as doctors treated his many shrapnel wounds and other serious injuries.(1) More than a century earlier, an aged Black Elk also cried when he wrote. “I did not know then how much was ended…I can still see the butchered women and children lying heaped and scattered all along the crooked gulch as plain as when I saw them with eyes still young.”(2) ... Brutal Civil War ... Global Manifest Destiny....
photo: AP / Associate Press File Photo
France opens WW2 Vichy regime files
Edit BBC News 28 Dec 2015
France opens up police and ministerial archives from the Vichy regime which helped Nazi Germany in World War Two ... ....
photo: Creative Commons / Flickr upload bot
In a first, Russia closes Red Square for New Year's eve
Edit The Times of India 28 Dec 2015
MOSCOW. Moscow's Red Square, traditionally a place where people gather to ring in the New Year, will be closed to revellers on December 31, city authorities said, amid mounting security concerns ... Speaking to Kommersant radio former lawmaker Alexander Klyukin said the surprise move was "the same as closing off Times Square in New York." ... ....
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PKU Original Drama "Good Morning, Mom" accomplishes 40 perfo... [2015-12-23] (PKU - Peking University)
Edit Public Technologies 23 Dec 2015
PKU - Peking University) The national tour also witnessed the growth of the drama team ... PKU - Peking University issued this content on 2015-12-23 and is solely responsible for the information contained herein ... Original Document. http.//english.pku.edu.cn/news_events/news/campus/4299.htm....
PKU and UNEP issue "The Report of the Model of Ecological We... [2015-12-23] (PKU - ...
Edit Public Technologies 23 Dec 2015
(Source. PKU - Peking University) On December 1, Paris Time, at the World Climate Conference in Paris, an 'ecological gift' from China is solemnly recommended by relevant institutions to the world ... PKU - Peking University issued this content on 2015-12-23 and is solely responsible for the information contained herein ... Original Document. http.//english.pku.edu.cn/news_events/news/global/4298.htm. (noodl. 31432858) ....
ICQM members report "Disorder and Metal-Insulator Transition... [2015-12-23] (PKU - Peking University)
Edit Public Technologies 23 Dec 2015
(Source. PKU - Peking University) Peking University, Dec. 22, 2015. A Ph.D.student Chen Chui-Zhenand his advisor Prof ... 115, 246603). This work was done in collaboration withPro. Song Juntao from Hebei Normal University, Prof ... Source ... PKU - Peking University issued this content on 2015-12-23 and is solely responsible for the information contained herein ... Original Document. http.//english.pku.edu.cn/news_events/news/research/4306.htm....
John Zacharias: ‘I have had such a wonderful time doing rese... [2015-12-23] (PKU - Peking ...
Edit Public Technologies 23 Dec 2015
(Source. PKU - Peking University) Peking University, Dec ... In 2012, professor Zacharias started his lab and began his full time tenure here at Peking University ... We know that you started doing research in China since 1993 ... Lai Huan ... PKU - Peking University issued this content on 2015-12-23 and is solely responsible for the information contained herein ... Original Document. http.//english.pku.edu.cn/news_events/news/people/4309.htm....
Press Conference Announcing the International Healthcare Service Session at the 4th China Beijing International Fair for Trade in Services
Edit PR Newswire 23 Dec 2015
BEIJING, Dec ... Representatives from chambers of commerce and foreign agencies stationed in China including the US, the UK, France, Belarus, Australia and South Korea as well as business executives from healthcare technology, insurance and investment companies, among them, PKU Healthcare, Baidu Healthcare, Xingshulin, Morgan Stanley, Bright Oceans Corporation, Yingzhi Healthcare and GL Capital Group, attended the press conference ... ....
Tianjin issues its first red alert for pollution, joining Beijing
Edit Mashable 22 Dec 2015
The city of Tianjin in China called its first ever red alert for air pollution on Monday night, joining Beijing, which called for its first two this month. Tianjin's red alert will last from midnight Wednesday to 6 a.m. Thursday. See also. Beijing's red alert is a sign that China must act on climate change ... #Tianjin #天津 #HighSpeedRailway #高铁 #China #Beijing #Peking #PekingUniversity #PKU #PKUExchange #KaillyoGoesPeking #KaillyoTravels ... ....
President of the University of Toronto visited Peking Univer... [2015-12-19] (PKU - Peking University)
Edit Public Technologies 19 Dec 2015
PKU - Peking University) President Gertler said that University of Toronto had achieved significant results in the aspect of international cooperation, and that the number of international students accounted for more than 20% of the total number of its students ... PKU News Center....
PKU and the UNEP issued “The Report of the Model of Ecologi... [2015-12-19] (PKU - ...
Edit Public Technologies 19 Dec 2015
(Source. PKU - Peking University) Peking University, Dec 16, 2015. On December 1, Paris Time, at the World Climate Conference in Paris, an 'ecological gift' from China is solemnly recommended by relevant institutions to the world ... PKU - Peking University issued this content on 2015-12-19 and is solely responsible for the information contained herein ... Original Document. http.//english.pku.edu.cn/news_events/news/global/4286.htm....
The New Year Poetry Recitation Conference and PKU Voluntary ... [2015-12-18] (PKU - Peking University) ...
Edit Public Technologies 18 Dec 2015
PKU - Peking University) On December 1, the 2016 New Year Recitation Conference of Chinese and Foreign Poetry - 'On Wings of Songs,' along with the opening ceremony of the 3rd PKU Voluntary Cultural Festival - 'PKU Voluntary Tree Action' was held in PKU Hall. Organized by Fanxing Poetry Society, PKU Communist ......
PKU student Zhang Wanyu won the 2015 Rhodes Scholar [2015-12-18] (PKU - Peking University)
Edit Public Technologies 18 Dec 2015
PKU - Peking University) Zhang Wanyu has gained a distinguished accomplishment in academic study, social practice, volunteer work and many other aspects ... In September of 2014, she was selected to join the student exchange program between PKU Law School and Stanford Law School, and was thought highly of by American teachers and students due to her excellent performance ... Zhang is an outstanding representative of PKU Law School student....
PKU Original Drama"Good Morning, Mom" Accomplished 40 Perfor... [2015-12-18] (PKU - Peking University)
Edit Public Technologies 18 Dec 2015
PKU - Peking University) The national tour also witnessed the growth of the drama team ... PKU - Peking University issued this content on 2015-12-18 and is solely responsible for the information contained herein ... Original Document. http.//english.pku.edu.cn/news_events/news/campus/4283.htm....
Yuan Ming: step from Peking University towards the world in ... [2015-12-18] (PKU - Peking ...
Edit Public Technologies 18 Dec 2015
PKU - Peking University) On November 24, PKU Office of International Relations kicked off the Training Sessions of Foreign Affairs of 2015 Fall Semester at the Sunshine Hall in Yingjie Overseas Exchange Center ... Youth in PKU ... 'What we can do' and 'what we should do' are the questions that every PKU student should reflect on. PKU is the paradise of campus life ... Professor Yuan Ming recalled the stories of outstanding PKU students....
Special ed teacher Rose Walsh dies at 80
Edit Chicago Sun-Times 17 Dec 2015
At 23, Rose Walsh was busy caring for her two babies, born 10 months apart in February and December of 1957. She was thrilled about starting a family ... The Walshes didn’t know it but the boys were born with PKU, a genetic disorder that inhibits the processing of protein. Children with Phenylketonuria must eat a low-protein diet, but Brian and Edmond were born before PKU screening of infants became mandatory in the U.S ... Her philosophy ... Mrs....
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