PSMD1


Description

The PSMD1 (proteasome 26S subunit, non-ATPase 1) is a protein-coding gene located on chromosome 2.

PSMD1, or 26S proteasome non-ATPase regulatory subunit 1, is a protein encoded by the PSMD1 gene in humans. It's one of 19 essential subunits contributing to the assembly of the 19S proteasome complex. The gene is located on chromosome 2q37.1 and consists of 25 exons. PSMD1 encodes the largest non-ATPase subunit of the 19S regulatory base, responsible for substrate recognition and binding. The human protein is 106 kDa in size, with 953 amino acids, and a theoretical pI of 5.25. An alternative splicing during gene expression can generate an isoform lacking amino acids 797-827. The 26S proteasome complex is composed of a 20S core particle (CP) and one or two 19S regulatory particles (RP). The CP has three proteolytic activities (caspase-like, trypsin-like, and chymotrypsin-like) located within its inner chamber, preventing random protein-enzyme encounters and uncontrolled protein degradation.

PSMD1 is a component of the 26S proteasome, a multiprotein complex responsible for degrading ubiquitinated proteins in an ATP-dependent manner. This process is crucial for maintaining protein homeostasis by removing misfolded or damaged proteins, ensuring proper cellular function, and eliminating proteins no longer needed. The proteasome plays a significant role in various cellular processes, including cell cycle regulation, apoptosis, and DNA damage repair.

PSMD1 is also known as P112, Rpn2, S1.

Associated Diseases



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