Recombinant Human Biogenesis of lysosome-related organelles complex 1 subunit 1 (BLOC1S1)

Uniprot Id

P78537

Target Species

Human

Target Name

BLOC1S1

Target Full Name

Biogenesis of lysosome-related organelles complex 1 subunit 1

Target Function

Component of the BLOC-1 complex, a complex that is required for normal biogenesis of lysosome-related organelles (LRO), such as platelet dense granules and melanosomes. In concert with the AP-3 complex, the BLOC-1 complex is required to target membrane protein cargos into vesicles assembled at cell bodies for delivery into neurites and nerve terminals. The BLOC-1 complex, in association with SNARE proteins, is also proposed to be involved in neurite extension. As part of the BORC complex may play a role in lysosomes movement and localization at the cell periphery. Associated with the cytosolic face of lysosomes, the BORC complex may recruit ARL8B and couple lysosomes to microtubule plus-end-directed kinesin motor.; May negatively regulate aerobic respiration through mitochondrial protein lysine-acetylation. May counteract the action of the deacetylase SIRT3 by acetylating and regulating proteins of the mitochondrial respiratory chain including ATP5F1A and NDUFA9.

Target Subcellular Location

Mitochondrion intermembrane space. Mitochondrion matrix. Cytoplasm, cytosol. Lysosome membrane.

Target Protein Families

BLOC1S1 family

Target Synonyms

BLOC1S1; BLOS1; GCN5L1; RT14Biogenesis of lysosome-related organelles complex 1 subunit 1; BLOC-1 subunit 1; GCN5-like protein 1; Protein RT14

Target Background

BLOC1S1 is a component of the ubiquitously expressed BLOC1 multisubunit protein complex. BLOC1 is required for normal biogenesis of specialized organelles of the endosomal-lysosomal system, such as melanosomes and platelet dense granules (Starcevic and Dell'Angelica, 2004 [PubMed 15102850]).