Page last updated: 2024-10-24

BLOC-1 complex

Definition

Target type: cellularcomponent

A protein complex required for the biogenesis of specialized organelles of the endosomal-lysosomal system, such as melanosomes and platelet dense granules. Many of the protein subunits are conserved between mouse and human; the mouse complex contains the Pallidin, Muted, Cappuccino, Dysbindin, Snapin, BLOS1, BLOS2, AND BLOS3 proteins. [PMID:15102850]

The Biogenesis of Lysosome-related Organelles Complex 1 (BLOC-1) is a multi-protein complex that plays a crucial role in the biogenesis of lysosome-related organelles (LROs). These organelles are specialized compartments within cells that perform a variety of functions, including the degradation of cellular waste, the storage and release of signaling molecules, and the defense against pathogens. BLOC-1 is essential for the proper formation and function of LROs, and mutations in BLOC-1 genes can lead to a variety of human diseases, including Hermansky-Pudlak syndrome (HPS) and Chediak-Higashi syndrome.

BLOC-1 is a large complex that is composed of eight subunits: BLOS1, BLOS2, BLOS3, BLOS4, BLOS5, BLOS6, BLOS7, and BLOS8. These subunits are arranged into three distinct modules: the BLOS1-BLOS2-BLOS3 module, the BLOS4-BLOS5 module, and the BLOS6-BLOS7-BLOS8 module.

The BLOS1-BLOS2-BLOS3 module is responsible for binding to the transmembrane protein AP-3, which is a key component of the machinery that sorts proteins to LROs. The BLOS4-BLOS5 module is thought to play a role in recruiting other proteins to the complex. The BLOS6-BLOS7-BLOS8 module is responsible for interacting with the Rab GTPase Rab32, which is a small GTP-binding protein that regulates the trafficking of LROs.

BLOC-1 is a multi-subunit complex that is essential for the proper biogenesis of lysosome-related organelles. It is composed of eight subunits that are arranged into three modules. Each module plays a distinct role in the function of the complex. Mutations in BLOC-1 genes can lead to a variety of human diseases.'
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Proteins (1)

ProteinDefinitionTaxonomy
Phosphatidylinositol 4-kinase type 2-alphaA phosphatidylinositol 4-kinase type 2-alpha that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q9BTU6]Homo sapiens (human)

Compounds (14)

CompoundDefinitionClassesRoles
9-ethyladenine
n(6),n(6)-dimethyladenineN(6),N(6)-dimethyladenine : A tertiary amine that is adenine substituted at N-6 by geminal methyl groups.tertiary amine
1-phenyl-4-pyrazolo[3,4-d]pyrimidinaminepyrazoles;
ring assembly
sq 225369-(tetrahydrofuryl)adenine : A nucleoside analogue that is adenine in which the nitrogen at position 9 has been substituted by a tetrahydrofuran-2-yl group. It is an adenylate cyclase inhibitor.nucleoside analogue;
oxolanes
EC 4.6.1.1 (adenylate cyclase) inhibitor
adenosine diphosphateAdenosine Diphosphate: Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.adenosine 5'-phosphate;
purine ribonucleoside 5'-diphosphate
fundamental metabolite;
human metabolite
2-aminopurine2-aminopurine : The parent compound of the 2-aminopurines, comprising a purine core carrying an amino substituent at the 2-position.

2-Aminopurine: A purine that is an isomer of ADENINE (6-aminopurine).

aminopurine : Any purine having at least one amino substituent.
2-aminopurines;
nucleobase analogue
antimetabolite
alpha-aminopyridinealpha-aminopyridine: RN given refers to parent cpd; structure in Merck Index, 9th ed, #485

aminopyridine : Compounds containing a pyridine skeleton substituted by one or more amine groups.
9-benzyladenine
adenosinequinquefolan B: isolated from roots of Panax quinquefolium L.; RN not in Chemline 10/87; RN from Toxlitadenosines;
purines D-ribonucleoside
analgesic;
anti-arrhythmia drug;
fundamental metabolite;
human metabolite;
vasodilator agent
benzylaminopurinebenzylaminopurine: a plant growth regulator

N-benzyladenine : A member of the class of 6-aminopurines that is adenine in which one of the hydrogens of the amino group is replaced by a benzyl group.
6-aminopurinescytokinin;
plant metabolite
9-methyladenine9-methyladenine : Adenine substituted with a methyl group at position N-9.methyladeninemetabolite
4-aminopyrazolo(3,4-d)pyrimidine4-aminopyrazolo(3,4-d)pyrimidine: adenine analog which suppresses growth of E coli & Bacillus cereus; inhibits cell growth & purine biosynthesis in rat hepatoma
4-aminoquinazoline4-aminoquinazoline: structure in first source
wortmanninacetate ester;
cyclic ketone;
delta-lactone;
organic heteropentacyclic compound
anticoronaviral agent;
antineoplastic agent;
autophagy inhibitor;
EC 2.7.1.137 (phosphatidylinositol 3-kinase) inhibitor;
geroprotector;
Penicillium metabolite;
radiosensitizing agent