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feline leukemia virus subgroup C receptor-related protein 1 (feline leukemia virus subgroup C receptor, hFLVCR, FLVCR1, FLVCR)
Function:
- isoform 1:
- exports cytoplasmic heme
- protection of developing erythroid cells from heme toxicity (putative)
- required during erythtopoiesis to maintain intracellular free heme balance since in proerythroblasts, heme synthesis intensifies & it's accumulation is toxic for cells
- protection from heme or Fe+2 toxicities in liver & brain
- exports coproporphyrin & protoporphyrin IX, both intermediates in the heme biosynthesis
- does not export bilirubin
- susceptibility to FeLV-C in vitro
- isoform 2
- heme transporter that promotes heme efflux from the mitochondrion to the cytoplasm
- essential for erythroid differentiation
- interacts with HPX
Structure:
- isoform 2 is N-glycosylated
- belongs to the major facilitator superfamily, Feline leukemia virus subgroup C receptor (TC 2.A.1.28.1) family
Compartment:
- multi-pass membrane protein
- isoform 1: cell membrane
- isoform 2: mitochondrial membrane
Alternative splicing:
- named isoforms=2
- probable mitochondrial transit peptide at positions 1-38
Expression:
- expressed in all hematopoietic tissues including: peripheral blood lymphocytes, fetal liver > pancreas, kidney
- down-regulated in hemopoietic progenitor cells undergoing differentiation & hemoglobinization
Pathology:
- defects in FLVCR1 are the cause of PCARP posterior column ataxia with retinitis pigmentosa
General
transmembrane 12 protein
Properties
SIZE: MW = 60 kD
entity length = 555 aa
COMPARTMENT: cellular membrane
MOTIF: transmembrane domain {108-128}
transmembrane domain {148-168}
transmembrane domain {175-195}
transmembrane domain {200-220}
transmembrane domain {241-261}
transmembrane domain {276-296}
transmembrane domain {332-352}
transmembrane domain {373-393}
transmembrane domain {402-422}
transmembrane domain {425-445}
transmembrane domain {460-480}
transmembrane domain {491-511}
Database Correlations
OMIM correlations
UniProt Q9Y5Y0
Pfam PF07690
Entrez Gene 28982
Kegg hsa:28982
References
UniProt :accession Q9Y5Y0