Information on EC 3.6.3.47 - fatty-acyl-CoA-transporting ATPase and Organism(s) Homo sapiens

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Homo sapiens


The taxonomic range for the selected organisms is: Homo sapiens

The enzyme appears in selected viruses and cellular organisms

EC NUMBER
COMMENTARY hide
3.6.3.47
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RECOMMENDED NAME
GeneOntology No.
fatty-acyl-CoA-transporting ATPase
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
hydrolysis of phosphoric ester
transmembrane transport
SYSTEMATIC NAME
IUBMB Comments
ATP phosphohydrolase (fatty-acyl-CoA-transporting)
ABC-type (ATP-binding cassette-type) ATPase, characterized by the presence of two similar ATP-binding domains. Does not undergo phosphorylation during the transport process. An animal and yeast enzyme that transports fatty acyl CoA into and out of peroxisomes. In humans, it is associated with Zellweger's syndrome.
CAS REGISTRY NUMBER
COMMENTARY hide
9000-83-3
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ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
physiological function
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ABC transporter ALDP interacts with ACLY and FATP4, ABC transporter PMP70 interacts with FASN and FATP4 involved in fatty acid metabolism, thus constituting a fatty acid synthesis-transport machinery at the cytoplasmic side of the peroxisomal membrane
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
ATP + H2O + fatty acyl CoA/cis
ADP + phosphate + fatty acyl CoA/trans
show the reaction diagram
additional information
?
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NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
ATP + H2O + fatty acyl CoA/cis
ADP + phosphate + fatty acyl CoA/trans
show the reaction diagram
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
Pex19p
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protein required for the peroxisomal membrane synthesis, binds to PMP70 co-translationally and keeps PMP70 in a proper conformation for the localization to peroxisome, PMP70 forms 75% aggregates during translation in the absence of Pex19p
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
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X-ALD fibroblast lacking ALDP
Manually annotated by BRENDA team
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SV40-transformed, from a patient with X-linked adrenoleukodystrophy missense mutation A626T
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
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when the N-terminal 80 amino acid residue (N80)-segment preceding transmembrane segment (TM) 1 is deleted and the TM1-TM2 region is fused to EGFP, the TM1 segment induces endoplasmic reticulum-targeting
Manually annotated by BRENDA team
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when the N-terminal 80-segment is fused to EGFP, the fusion protein is targeted to the outer mitochondrial membrane
Manually annotated by BRENDA team
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
75000
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monomer, SDS-PAGE and immunoblotting
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
into pECFP-N1 and pEYFP-N1 vectors for transfection of VERO cells; into pECFP-N1, pEYFP-N1, or pECFP-C1 vectors for transfection of VERO cells
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into the pMAM2-BSD, pEGFPN-1 and pcDNA4HisMax TOP vector for transfection of CHO cells, and as templates for the construction of mutant cDNAs
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using transient transfection of COS cells
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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
ALDP390
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mutant, comprises the amino acids 1 to 390
ALDP550
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mutant, comprises the amino acids 1 to 550
ALDP658
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mutant, comprises the amino acids 1 to 658
ALDPDELTA551-657
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mutant, residues 551-657 are deleted
ALDPDELTANBF
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mutant, residues 391-657 are deleted
G116R
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naturally occuring missense mutation
H667D
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naturally occuring missense mutation
Q544R
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naturally occuring missense mutation
R104C
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naturally occuring missense mutation
R617H
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naturally occuring missense mutation
S342P
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naturally occuring missense mutation
S606L
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naturally occuring missense mutation
S606P
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naturally occuring missense mutation
Y174C
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naturally occuring missense mutation
additional information
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine