OBSL1 : obscurin like cytoskeletal adaptor 1


Description

The OBSL1 (obscurin like cytoskeletal adaptor 1) is a protein-coding gene located on chromosome 2.

The OBSL1 gene provides instructions for making a protein that is thought to help maintain normal levels of another protein called cullin-7, which is produced from the CUL7 gene. Cullin-7 plays a role in the ubiquitin-proteasome system, which is the cell machinery that breaks down (degrades) unwanted proteins. Cullin-7 helps assemble a complex known as an E3 ubiquitin ligase. This complex tags damaged and excess proteins with molecules called ubiquitin. Ubiquitin serves as a signal to specialized cell structures known as proteasomes, which attach (bind) to the tagged proteins and degrade them. The ubiquitin-proteasome system acts as the cell‘s quality control system by disposing of damaged, misshapen, and excess proteins. This system also regulates the level of proteins involved in several critical cell activities such as the timing of cell division and growth. In particular, the OBSL1 protein and cullin-7 are thought to help regulate proteins involved in the body‘s response to growth hormones, although their specific role in this process is unknown.

OBSL1 plays a crucial role in regulating microtubule dynamics and genome integrity as a core component of the 3M complex. The precise mechanism of 3M complex function in microtubule regulation is unknown, but it may involve controlling the levels of microtubule stabilizers. OBSL1 also acts as a regulator of the Cul7-RING(FBXW8) ubiquitin-protein ligase, contributing significantly to the ubiquitin ligase pathway that governs Golgi morphogenesis and dendrite patterning in the brain. Its function in neuronal Golgi apparatus localization of CUL7 is essential.

OBSL1 is also known as -.

Associated Diseases


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