ABCD3 - ATP-binding cassette sub-family D member 3 - human protein (Function)
 
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Gene
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ABCD3 »  ATP-binding cassette sub-family D member 3
 
Protein also known as:  70 kDa peroxisomal membrane protein (PMP70).
Gene name:  ABCD3
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 56 3
GENE REF ISO

Function

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Overview 
Probable transporter involved in the transport of branched-chain fatty acids and C27 bile acids into the peroxisome; the latter function is a crucial step in bile acid biosynthesis (PubMed25168382). The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity (PubMed11248239).  
3
  • UniProtKB
According to TCDB this is a transporter from family:
the atp-binding cassette (abc) superfamily  3.A.1.203.1  
GO molecular function 
ATP bindingdefinition[GO:0005524]  
1
  • UniProtKB
ATPase activitydefinition[GO:0016887]  
1
  • UniProtKB
ATPase activity, coupled to transmembrane movement of substancesdefinition[GO:0042626] silver  
1
  • InterPro 2 GO
Long-chain fatty acid transporter activitydefinition[GO:0005324] silver  
1
  • InterPro 2 GO
Protein bindingdefinition[GO:0005515]  
10
  • IntAct
  • UniProtKB
Protein homodimerization activitydefinition[GO:0042803]  
1
  • UniProtKB
Protein self-associationdefinition[GO:0043621] silver  
1
  • Ortholog Compara
GO biological process 
Fatty acid beta-oxidationdefinition[GO:0006635]  
1
  • UniProtKB
Fatty acid biosynthetic processdefinition[GO:0006633]  
1
  • UniProtKB
Peroxisomal long-chain fatty acid importdefinition[GO:0015910] silver  
1
  • InterPro 2 GO
Peroxisome organizationdefinition[GO:0007031]  
2
  • UniProtKB
Response to drugdefinition[GO:0042493] silver  
1
  • Ortholog Compara
Response to organic cyclic compounddefinition[GO:0014070] silver  
1
  • Ortholog Compara
Very long-chain fatty acid catabolic processdefinition[GO:0042760]  
1
  • UniProtKB
Pathways 
According to KEGG, this protein belongs to the following pathways:
ABC transporters  hsa02010+5825  
Peroxisome  hsa04146+5825  
According to Reactome, this protein belongs to the following pathway:
ABC transporters in lipid homeostasis  R-HSA-1369062  
Note 
Mutation in ABCD3 have been found in two individuals affected by Zellweger syndrome. However, the role of ABCD3 in the causation of Zellweger syndrome remains uncertain.  
1
  • UniProtKB
 

Keywords

Biological process 
Transport  definition   [KW-0813]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

GeneWiki: ABCD3
GenomeRNAi: 5825
PRO: PR:P28288