ID   HPS1_MACFA              Reviewed;         667 AA.
AC   Q60HF3;
DT   29-MAR-2005, integrated into UniProtKB/Swiss-Prot.
DT   23-NOV-2004, sequence version 1.
DT   03-AUG-2022, entry version 32.
DE   RecName: Full=BLOC-3 complex member HPS1 {ECO:0000305};
DE   AltName: Full=Hermansky-Pudlak syndrome 1 protein homolog;
GN   Name=HPS1; ORFNames=QflA-21346;
OS   Macaca fascicularis (Crab-eating macaque) (Cynomolgus monkey).
OC   Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC   Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini;
OC   Cercopithecidae; Cercopithecinae; Macaca.
OX   NCBI_TaxID=9541;
RN   [1]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC   TISSUE=Frontal cortex;
RA   Kusuda J., Osada N., Tanuma R., Hirata M., Sugano S., Hashimoto K.;
RT   "Isolation and characterization of cDNA for macaque neurological disease
RT   genes.";
RL   Submitted (OCT-2003) to the EMBL/GenBank/DDBJ databases.
CC   -!- FUNCTION: Component of the BLOC-3 complex, a complex that acts as a
CC       guanine exchange factor (GEF) for RAB32 and RAB38, promotes the
CC       exchange of GDP to GTP, converting them from an inactive GDP-bound form
CC       into an active GTP-bound form. The BLOC-3 complex plays an important
CC       role in the control of melanin production and melanosome biogenesis and
CC       promotes the membrane localization of RAB32 and RAB38.
CC       {ECO:0000250|UniProtKB:Q92902}.
CC   -!- SUBUNIT: Component of the biogenesis of lysosome-related organelles
CC       complex-3 (or BLOC-3), a heterodimer of HPS1 and HPS4. HPS1 cannot but
CC       BLOC-3 complex (heterodimer of HPS1 and HPS4) can interact with the
CC       GTP-bound form of RAB9A and RAB9B. HPS1 and BLOC-3 complex do not
CC       interact with the GDP-bound form of RAB9A and RAB9B.
CC       {ECO:0000250|UniProtKB:Q92902}.
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DR   EMBL; AB125174; BAD51962.1; -; mRNA.
DR   AlphaFoldDB; Q60HF3; -.
DR   STRING; 9541.XP_005566208.1; -.
DR   eggNOG; ENOG502QW8U; Eukaryota.
DR   Proteomes; UP000233100; Unplaced.
DR   GO; GO:0031085; C:BLOC-3 complex; ISS:UniProtKB.
DR   GO; GO:0005085; F:guanyl-nucleotide exchange factor activity; IEA:UniProtKB-KW.
DR   GO; GO:1903232; P:melanosome assembly; ISS:UniProtKB.
DR   GO; GO:0016192; P:vesicle-mediated transport; IEA:InterPro.
DR   InterPro; IPR043972; FUZ/MON1/HPS1_longin_1.
DR   InterPro; IPR043971; FUZ/MON1/HPS1_longin_2.
DR   InterPro; IPR043970; FUZ/MON1/HPS1_longin_3.
DR   InterPro; IPR026053; HPS1.
DR   PANTHER; PTHR12761; PTHR12761; 1.
DR   Pfam; PF19036; Fuz_longin_1; 1.
DR   Pfam; PF19037; Fuz_longin_2; 1.
DR   Pfam; PF19038; Fuz_longin_3; 1.
PE   2: Evidence at transcript level;
KW   Guanine-nucleotide releasing factor; Reference proteome; Repeat.
FT   CHAIN           1..667
FT                   /note="BLOC-3 complex member HPS1"
FT                   /id="PRO_0000084048"
FT   REPEAT          45..51
FT                   /note="[DE]-X(4)-L-L 1"
FT   REPEAT          147..153
FT                   /note="[DE]-X(4)-L-L 2"
FT   REPEAT          483..489
FT                   /note="[DE]-X(4)-L-L 3"
FT   REPEAT          611..617
FT                   /note="[DE]-X(4)-L-L 4"
FT   MOTIF           665..667
FT                   /note="Melanosome targeting signal"
FT                   /evidence="ECO:0000255"
SQ   SEQUENCE   667 AA;  75711 MW;  739492AA58601F8B CRC64;
     MKCVLVATEG AEVLFYWTDD EFEESLRLKF GQSENEEEEL PALEDQLSTL LAPVIISSMT
     MLEKLSDTYT CFSTENGNSL YVLHLFGECL FIAINGDHTE NEGDLRRKLY VLKYLFEVHF
     GLVTVDGQLI RKELRPPDLG QRVQLWEHFQ SLLWTYSRLR EQEQCFAVEA LERLIHPQLC
     ELCIEALERH VIQAVNTSSE RGGEEALHAF LLVHSKLLAF YSSHSASSLR PADLLVLILL
     VQDLYPSENT VEDDTQETDS FSLPEEYFTP APSPGDQSSG STIWLEGGTP PMEALQIAED
     TLQTLVPHCP APSSPRRIFL DANVKESYCP LVPHTMYCLP LWPGINLVLL TRSPSAPLAL
     VLSQLMDGFA MLEKKLKEGP EAGASLRSQP LVGDLRQRMD KFVKNRGAQE IQSTWLEFKA
     KAFSKSEPGS SSELLQACGK LKRQLCAIYR LNFLTTAPNR GGPHLPQHLQ DQVQRLMREK
     LTDWKDFLLV KSRRNITMVS YLEDFPGLVH FIYVDRTTGQ MVAPSLNCSE KTSSELGKGP
     LAAFVKTKVW SLIQLARRYL QKGYTTLLFQ EGDFYCSYFL WFENDMGYKL QMIEVPVLSD
     DSVPIGMLGG DYYRKLLRYY SKNRPAEAVR CYELLALHLS VIPTDLLVQQ AGQLARRLWE
     ASRIPLL