Protein detail

HPS4

BLOC-3 complex member HPS4 (Hermansky-Pudlak syndrome 4 protein) (Light-ear protein homolog)

Entry name
HPS4
UniProt ID
EVMP score
0.38
Frequency
4
Transmembrane count
Protein classification
Disease related genesHuman disease related genesPredicted intracellular proteins
EVMP score: annotation confidence score.
Extremely high >= 0.85High >= 0.70Medium >= 0.55Low >= 0.40
Basic Information
Protein Names
BLOC-3 complex member HPS4 (Hermansky-Pudlak syndrome 4 protein) (Light-ear protein homolog)
Protein Class
Disease related genesHuman disease related genesPredicted intracellular proteins
Protein Function
  • Disease related genes
  • Predicted intracellular proteins
  • Human disease related genes:Congenital disorders of metabolism:Congenital disorders of amino acid metabolism
Entrez Gene Symbol
Gene Synonym
BLOC3S2KIAA1667LE
Gene Description
HPS4 biogenesis of lysosomal organelles complex 3 subunit 2
Chromosome
22
Position
26443107-26483931
Frequency
4
EVMP Score
0.38
Fluorescence & Localization
HPS4 fluorescence
Tissue Specificadrenal glandCell SpecificBreast lactating cellsSingle-Nuclei Brain Specificendothelial cell
Function & Pathway
Relations & Evidence

Enzyme-Mediated Modification

0 records.

Ligand-Receptor Signaling

4 records.

CategoryParentDatabaseTransmitterReceiverSecretedPlasma Membrane (Transmembrane)Plasma Membrane (Peripheral)
intracellularintracellularLOCATENoNoNoNoNo
intracellularintracellularComPPINoNoNoNoNo
intracellularintracellularGO_IntercellNoNoNoNoNo
intracellularintracellularOmniPathNoNoNoNoNo

Regulatory Interaction Network

0 records.

Protein Complex Composition

1 record.

Component NameComponent Gene SymbolsComponent UniProt IDStoichiometryDatabaseDatabase IDsReferences
BLOC-3HPS1HPS4Q92902Q9NQG71:1CORUMSIGNORCompleatComplexPortalintact:EBI-22228063SIGNOR:SIGNOR-C253CORUM:11Compleat:HC1348303879132324740512756248231743012372602214755292161852711284729020048159

Isolation & Detection Technology

1 record.

EV Isolation MethodDetection MethodNumber of ReferencesReferences
Differential UltracentrifugationSize Exclusion ChromatographyMass spectrometry140222457
Sequence, Structure & Domains

Sequences

Length
708
Mass
76,919
Sequence
MATSTSTEAKSASWWNYFFLYDGSKVKEEGDPTRAGICYFYPSQTLLDQQELLCGQIAGVVRCVSDISDSPPTLVRLRKLKFAIKVDGDYLWVLGCAVELPDVSCKRFLDQLVGFFNFYNGPVSLAYENCSQEELSTEWDTFIEQILKNTSDLHKIFNSLWNLDQTKVEPLLLLKAARILQTCQRSPHILAGCILYKGLIVSTQLPPSLTAKVLLHRTAPQEQRLPTGEDAPQEHGAALPPNVQIIPVFVTKEEAISLHEFPVEQMTRSLASPAGLQDGSAQHHPKGGSTSALKENATGHVESMAWTTPDPTSPDEACPDGRKENGCLSGHDLESIRPAGLHNSARGEVLGLSSSLGKELVFLQEELDLSEIHIPEAQEVEMASGHFAFLHVPVPDGRAPYCKASLSASSSLEPTPPEDTAISSLRPPSAPEMLTQHGAQEQLEDHPGHSSQAPIPRADPLPRRTRRPLLLPRLDPGQRGNKLPTGEQGLDEDVDGVCESHAAPGLECSSGSANCQGAGPSADGISSRLTPAESCMGLVRMNLYTHCVKGLVLSLLAEEPLLGDSAAIEEVYHSSLASLNGLEVHLKETLPRDEAASTSSTYNFTHYDRIQSLLMANLPQVATPQDRRFLQAVSLMHSEFAQLPALYEMTVRNASTAVYACCNPIQETYFQQLAPAARSSGFPNPQDGAFSLSGKAKQKLLKHGVNLL
Alternative Products
Event=Alternative splicing; Named isoforms=4; Name=1; IsoId=Q9NQG7-1; Sequence=Displayed; Name=2; IsoId=Q9NQG7-2; Sequence=VSP_003880, VSP_003881, VSP_003882; Name=3; IsoId=Q9NQG7-3; Sequence=VSP_017082; Name=4; IsoId=Q9NQG7-4; Sequence=VSP_017082, VSP_003880
Alternative Sequence
1..14; MATSTSTEAKSASW -> MAPLCSLAR (in isoform 3 and isoform 4); 236; G -> GKWMLWSFKNRVTHQNPNG (in isoform 2 and isoform 4); 463..510; RRTRRPLLLPRLDPGQRGNKLPTGEQGLDEDVDGVCESHAAPGLECSS -> KSTVLFSGGCVKGSDTQLCVPGLVWYLEPQFYYVIVRHFFDSMRQTAG (in isoform 2); 511..708; Missing (in isoform 2)

3D Structural Models

Domain & Motif Annotations

Compositional Bias
319..331; Basic and acidic residues
Region
269..331; Disordered; 407..490; Disordered
Clinical Relevance
Disease Involvement
AlbinismHermansky-Pudlak syndrome