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ATP-binding cassette sub-family D member 3 (70 kD peroxisomal membrane protein, ABCD3, PMP70, PXMP1)
Function:
- probable transporter
- the nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity
- can form heterodimers with ABCD1/ALD & ABCD2/ALDR
- dimerization is necessary to form an active transporter
- interacts with PEX19
Structure:
- belongs to the ABC transporter family, ALD subfamily
- contains 1 ABC transmembrane type-1 domain
- contains 1 ABC transporter domain
Compartment: peroxisome membrane
Alternative splicing: named isoforms=3
Pathology:
- defects in ABCD3 may be the cause of Zellweger syndrome type 2
General
ATP-binding cassette sub-family D (ABC transporter-D, ABCD)
glycoprotein
Properties
SIZE: entity length = 659 aa
MW = 75 kD
COMPARTMENT: peroxisome
MOTIF: PEX19 interaction {1-199}
MOTIF: Targeting to peroxisomes {1-124}
N-glycosylation site {N12}
transmembrane domain {84-104}
ABC transmembrane type-1 {85-372}
MOTIF: N-glycosylation site {N106}
transmembrane domain {126-146}
Tyr phosphorylation site {Y145}
N-glycosylation site {N206}
transmembrane domain {224-244}
transmembrane domain {313-333}
ABC transporter {440-659}
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 473-480
Database Correlations
OMIM 170995
UniProt P28288
PFAM correlations
Entrez Gene 5825
Kegg hsa:5825
References
UniProt :accession P28288