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)
| Protein | Definition | Taxonomy |
|---|---|---|
| Phosphatidylinositol 4-kinase type 2-alpha | A phosphatidylinositol 4-kinase type 2-alpha that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q9BTU6] | Homo sapiens (human) |
Compounds (14)
| Compound | Definition | Classes | Roles |
|---|---|---|---|
| 9-ethyladenine | |||
| n(6),n(6)-dimethyladenine | N(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]pyrimidinamine | pyrazoles; ring assembly | ||
| sq 22536 | 9-(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 diphosphate | Adenosine 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-aminopurine | 2-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-aminopyridine | alpha-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 | |||
| adenosine | quinquefolan B: isolated from roots of Panax quinquefolium L.; RN not in Chemline 10/87; RN from Toxlit | adenosines; purines D-ribonucleoside | analgesic; anti-arrhythmia drug; fundamental metabolite; human metabolite; vasodilator agent |
| benzylaminopurine | benzylaminopurine: 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-aminopurines | cytokinin; plant metabolite |
| 9-methyladenine | 9-methyladenine : Adenine substituted with a methyl group at position N-9. | methyladenine | metabolite |
| 4-aminopyrazolo(3,4-d)pyrimidine | 4-aminopyrazolo(3,4-d)pyrimidine: adenine analog which suppresses growth of E coli & Bacillus cereus; inhibits cell growth & purine biosynthesis in rat hepatoma | ||
| 4-aminoquinazoline | 4-aminoquinazoline: structure in first source | ||
| wortmannin | acetate 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 |