Ubiquitin carboxy-terminal hydrolase L1
Ubiquitin carboxy-terminal hydrolase L1 (EC 3.1.2.15, ubiquitin C-terminal hydrolase, UCH-L1) is a deubiquitinating enzyme.
Function
UCH-L1 is a member of a gene family whose products hydrolyze small C-terminal adducts of ubiquitin to generate the ubiquitin monomer. Expression of UCH-L1 is highly specific to neurons and to cells of the diffuse neuroendocrine system and their tumors. It is abundantly present in all neurons (accounts for 1-2% of total brain protein), expressed specifically in neurons and testis/ovary.
The catalytic triad of UCH-L1 contains a cysteine at position 90, an aspartate at position 176, and a histidine at position 161 that are responsible for its hydrolase activity.
Relevance to neurodegenerative disorders
A point mutation (I93M) in the gene encoding this protein is implicated as the cause of Parkinson's disease in one German family, although this finding is controversial, as no other Parkinson's disease patients with this mutation have been found.
Podcasts:
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by Glenn Hughes
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by Peter Frampton
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by La Ley
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by Hot Rod Circuit
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by Anniversary
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by Paper Route
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by Diafanes
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by Silencers
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by Mike Keneally
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by Epoxies
You Kill Me
by: Glenn HughesIn my brain it's understood
You killed my soul like I knew you would
You hit me with your velvet touch
My sacrifice was all too much
Why did you have to walk away
On sacred ground yesterday
Fill my pride, you don't wanna know
Still inside, every time you go
You kill me
You took the breath right out of me
And now I find that I can't see
The shallow water is my grave
Now what I lost is what I crave
Fill my pride, you don't wanna know
Still inside, every time you go
You kill me
Now I can feel the ghost within
Don't put the curse on me again
Don't leave me in this golden cage
Until I live the final page
Fill my pride, you don't wanna know
Still inside, every time you go
You kill me
Fill my pride, you don't wanna know
Still inside, every time you go