UCH-L1 , PARK5 (4p13)

Tausta tieto:
 Mihin ryhmään ubikitiiniin vaikuttava entsyymi UCHL1 kuuluu?: 

Tämän UCHL1 geenin (4p13)  koodaman proteiinin ryhmäkuuluvuus on
 DUB, deubikitinaasit  
Deubikitinaaseja on satakunta ja niillä on 5 alaperhettä. Niisä on paljon  vaikutus resursseja tulevaisiin aikoihin asti, kun niitä saa kartoitetuksu. UCHL1  heräsi eloon uudestaan  viime vuosina ja kuten 2018 aikaan  siitä on tullut paljon tuoretta tietoa, jota voi hyödyntöä koska  sen expressio on vahvasti neuronaalinen, aivo. 

DUB- Alaperheet ovat:
USP (57 kpl),Ubiquitin Specific  proteases

UCH (4 kpl) , Ubiquitin C-terminal Hydrolases
OTUS (14) , Ovarian Tumor proteases
MJD (4) , Machado-Joseph-Disease protease, SCA3
JAMM (11), Jab1/Pad1/MPN-domain containig metalloenzymes 


UCH-joukko on  seuraava:
BAP1
UCH-L1
UCH-L3
UCH-L5 (UCH37; CGI-70)
Siis nyt katson UCH-L1  deubikitinaasista enemmän tietoa:

UCH-L1(4p13)

https://www.ncbi.nlm.nih.gov/pubmed/30788389
 https://www.ncbi.nlm.nih.gov/gene/7345

Also known as

NDGOA; PARK5; PGP95; SPG79; PGP9.5; Uch-L1; HEL-117; PGP 9.5; HEL-S-53

Summary

The protein encoded by this gene belongs to the peptidase C12 family. This enzyme is a thiol protease that hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. This gene is specifically expressed in the neurons and in cells of the diffuse neuroendocrine system. Mutations in this gene may be associated with Parkinson disease.[provided by RefSeq, Sep 2009]

Expression

Biased expression in brain (RPKM 389.7), adrenal (RPKM 70.8) and 2 other tissues See more Orthologs mouse all

Preferred Names

ubiquitin carboxyl-terminal hydrolase isozyme L1

Names

epididymis luminal protein 117

epididymis secretory protein Li 53

neuron cytoplasmic protein 9.5

ubiquitin carboxyl-terminal esterase L1 (ubiquitin thiolesterase)

ubiquitin thioesterase L1

ubiquitin thiolesterase

NP_004172.2

• EC 3.4.19.12

Related articles in PubMed

1. The deubiquitinating enzyme UCHL1 is a favorable prognostic marker in neuroblastoma as it promotes neuronal differentiation. Gu Y, et al. J Exp Clin Cancer Res, 2018 Oct 25. PMID 30359286, Free PMC Article
2. UCHL1/PGP 9.5 Dynamic in Neuro-Immune-Cutaneous Milieu: Focusing on Axonal Nerve Terminals and Epidermal Keratinocytes in Psoriatic Itch. Kupczyk P, et al. Biomed Res Int, 2018. PMID 30148172, Free PMC Article
3. Upregulation of Ubiquitin Carboxyl-Terminal Hydrolase L1 (UCHL1) Mediates the Reversal Effect of Verapamil on Chemo-Resistance to Adriamycin of Hepatocellular Carcinoma. Yang G, et al. Med Sci Monit, 2018 Apr 8. PMID 29627846, Free PMC Article
4. High Expression of Ubiquitin C-terminal Hydrolase L1 Is Associated With Poor Prognosis in Endometrial Cancer Patients. Nakao K, et al. Int J Gynecol Cancer, 2018 May. PMID 29489474
5. Ubiquitin C-Terminal Hydrolase L1 regulates autophagy by inhibiting autophagosome formation through its deubiquitinating enzyme activity. Yan C, et al. Biochem Biophys Res Commun, 2018 Mar 4. PMID 29462615

See all (275) citations in PubMed

See citations in PubMed for homologs of this gene provided by HomoloGene

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

1. high expression is a strong marker of poor prognosis of endometrial cancer
2. these results identified UCHL1 as a target podocyte protein of autoantibodies in a set of relapsing patients and support a causative role of anti-UCHL1 autoantibodies in the development of idiopathic steroid sensitive nephrotic syndrome
3. serum level not significantly elevated in newborns at risk for hypoxic ischemic encephalopathy but with normal EEGs
4. observed significantly altered density of PGP 9.5-positive axonal nerve terminals in pruritic lesional and nonlesional psoriatic skin compared with controls;PGP 9.5 expression by suprabasal keratinocytes (SBKs) was significantly increased in itchy skin lesions compared to skin without itch.
5. This study demonstrated that UCHL1 expression is induced by HCV infection. UCHL1 stimulates JNK phosphorylation and signaling related to HSCs activation after binding to the cell surface of HSCs.
6. Study showed that UCHL1 could serve as a prognostic marker indicating a favorable clinical outcome in Neuroblastoma (NB). UCHL1 expression was higher in ganglioneuroblastomas/ganglioneuromas (GNB/ GN) and well-differentiated NB than poorly differentiated NB, and UCHL1 expression was strongly associated with differentiation markers, indicating the positive correlation between UCHL1 expression and NB differentiation.
7. The proteins UCH-L1 and alpha-internexin could be independent prognostic biomarkers of pancreatic neuroendocrine tumors.
8. We have also demonstrated that UCHL1 S-nitrosylation provides seeding for faster aggregation of a-synuclein. Finally, the in vitro nitrosylation of UCHL1 was corroborated with rotenone induced mouse model of PD
9. In newborns undergoing cardiac surgery, ubiquitin C-terminal hydrolase 1 showed a significant rise at 0 hours in the deep hypothermic circulatory arrest group compared to baseline. Levels returned to baseline at 12 hours. There was an early rise in UCHL1 at 0 hours in the cardiopulmonary bypass group.
10. Overexpression of UCHL1 genes promoted apoptosis in cells treated with VER+ADM. UCHL1 knockdown using siRNA weakened the effect of ADM+VER, indicating that ADM+VER promotes HCC cell apoptosis and that UCHL1 genes participate in VER-mediated promotion in tumor cell apoptosis.

. Structure: History

https://www.ncbi.nlm.nih.gov/protein/NP_004172.2