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Proteolytic degradation is critical to the maintenance of appropriate levels of short-lived and regulatory proteins as important and diverse as those involved in cellular metabolism, heat shock and stress response, antigen presentation, modulation of cell surface receptors and ion channels, cell cycle regulation, transcription, and signalling factors. The ubiquitin-proteasome pathway deconstructs most proteins in the eukaryotic cell cytosol and nucleus. Others are degraded via the vacuolar pathway which includes endosomes, lysosomes, and the endoplasmic reticulum. The 26S proteasome is an ATP-dependent, multisubunit (approximately 31), barrel-shaped molecular machine with an apparent molecular weight of approximately 2.5 MDa. It consists of a 20S proteolytic core complex which is crowned at one or both ends by 19S regulatory subunit complexes. The 19S regulatory subunits recognize ubiquitinated proteins and play an essential role in unfolding and translocating targets into the lumen of the 20S subunit. An enzymatic cascade is responsible for the attachment of multiple ubiquitin molecules to lysine residues of proteins targeted for degradation. Several genetic diseases are associated with defects in the ubiquitin-proteasome pathway. Some examples of affected proteins include those linked to cystic fibrosis, Angelman's syndrome, and Liddle syndrome.
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蛋白别名: 11S regulator complex subunit alpha; 29-kD MCP activator subunit; Activator of multicatalytic protease subunit 1; epididymis secretory sperm binding protein Li 129m; IGUP I-5111; Interferon gamma up-regulated I-5111 protein; interferon-gamma IEF SSP 5111; interferon-gamma-inducible protein 5111; MGC8628; OTTHUMP00000164702; PA28 alpha subunit; PA28a; PA28alpha; protease (prosome, macropain) 28 subunit, alpha; proteasome (prosome, macropain) 28 subunit, alpha; proteasome (prosome, macropain) activator subunit 1 (PA28 alpha); Proteasome activator 28 subunit alpha; Proteasome activator complex subunit 1; REG-alpha
基因别名: AW413925; HEL-S-129m; IFI5111; PA28A; PA28alpha; PSME1; REGalpha
UniProt ID: (Human) Q06323, (Mouse) P97371
Entrez Gene ID: (Human) 5720, (Mouse) 19186