background:
The peroxisomal membrane contains several ATP-binding cassette (ABC) transporters, ABCD1-4 that are known to be present in the human peroxisome membrane. All four proteins are ABC half-transporters, which dimerize to form an active transporter. A mutation in the ABCD1 gene causes X-linked adreno-leukodystrophy (X-ALD), a peroxisomal disorder which affects lipid storage. ABCD2 in mouse is expressed at high levels in the brain and adrenal organs, which are adversely affected in X-ALD. The peroxisomal membrane comprises two quantitatively major proteins, PMP22 and ABCD3. ABCD3 is associated with irregularly shaped vesicles which may be defective peroxisomes or peroxisome precursors. ABCD1 localizes to peroxisomes. ABCB7 is a half-transporter involved in the transport of heme from the mitochondria to the cytosol.
Function:
Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.
Subunit:
Can form heterodimers with ABCD1/ALD and ABCD2/ALDR. Dimerization is necessary to form an active transporter. Interacts with PEX19.
Subcellular Location:
Peroxisome membrane; Multi-pass membrane protein.
Similarity:
Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily.
Contains 1 ABC transmembrane type-1 domain.
Contains 1 ABC transporter domain.
Database links:
Entrez Gene: 100765314 Hamster
Entrez Gene: 5825 Human
Entrez Gene: 19299 Mouse
Entrez Gene: 25270 Rat
Omim: 170995 Human
SwissProt: P28288 Human
SwissProt: P55096 Mouse
SwissProt: P16970 Rat
Unigene: 700576 Human
Unigene: 399042 Mouse
Unigene: 7024 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
|
|
