UBL4A
Description
The UBL4A (ubiquitin like 4A) is a protein-coding gene located on chromosome X.
Ubiquitin-like protein 4A is a protein that in humans is encoded by the UBL4A gene.
UBL4A, also known as Ubiquitin-like protein GDX, is a component of the BAG6/BAT3 complex, a cytosolic protein quality control complex. The BAG6/BAT3 complex plays a crucial role in maintaining misfolded and hydrophobic proteins in a soluble state, facilitating their delivery to the endoplasmic reticulum (ER) or alternatively promoting their sorting to the proteasome for degradation. This complex is involved in the post-translational delivery of tail-anchored/type II transmembrane proteins to the ER membrane. Upon recruiting to ribosomes, it interacts with the transmembrane region of newly synthesized tail-anchored proteins, and alongside SGTA and ASNA1, mediates their delivery to the ER. Client proteins that fail to be properly delivered to the ER are ubiquitinated and sorted to the proteasome. Furthermore, the BAG6/BAT3 complex acts as a sorting platform for mislocalized proteins of the secretory pathway, directing them either to the proteasome for degradation or back to the ER. This complex also participates in ER-associated degradation (ERAD), a quality control mechanism that eliminates unwanted ER proteins by retrotranslocating them to the cytosol and targeting them to the proteasome. It maintains these retrotranslocated proteins in an unfolded yet soluble state within the cytosol, ensuring their proper delivery to the proteasome.
UBL4A is also known as DX254E, DXS254E, G6PD, GDX, GET5, MDY2, TMA24, UBL4.
Associated Diseases
- pancreatic ductal adenocarcinoma
- cancer
- acute kidney failure
- skeletal dysplasia
- Melnick-Needles syndrome
- SATB2 associated disorder
