Recombinant Mouse ATP-binding cassette sub-family D member 1 (Abcd1), partial

Product Details

Purity
>85% (SDS-PAGE)
Target Names
Abcd1
Uniprot NO.
Alternative Names
Abcd1; Ald; Aldgh; ATP-binding cassette sub-family D member 1; Adrenoleukodystrophy protein; ALDP
Species
Mus musculus (Mouse)
Source
Yeast
Protein Length
Partial
Tag Info
N-terminal His-tagged/Tag-Free
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Storage Condition
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

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Target Background

Function(From Uniprot)
ATP-dependent transporter of the ATP-binding cassette (ABC) family involved in the transport of very long chain fatty acid (VLCFA)-CoA from the cytosol to the peroxisome lumen. Has fatty acyl-CoA thioesterase (ACOT) and ATPase activities. Coupled to the ATP-dependent transporter activity has also a fatty acyl-CoA thioesterase activity (ACOT) and hydrolyzes VLCFA-CoA into VLCFA prior their ATP-dependent transport into peroxisomes, the ACOT activity is essential during this transport process. Thus, plays a role in regulation of VLCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation, mitochondrial function and microsomal fatty acid elongation. Involved in several processes; namely, controls the active myelination phase by negatively regulating the microsomal fatty acid elongation activity and may also play a role in axon and myelin maintenance. Controls also the cellular response to oxidative stress by regulating mitochondrial functions such as mitochondrial oxidative phosphorylation and depolarization. And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs.
Gene References into Functions
  1. ABCD1 and its homolog ABCD2 exist mainly as homotetramers in the peroxisomal membrane PMID:28258215
  2. during the active myelination phase the microsomal fatty acid elongation activity is stimulated in abcd1-deficient mice PMID:26108493
  3. Deletion of AMPKalpha1 in the mixed glial cells of Abcd1-KO mice induced spontaneous mitochondrial dysfunction PMID:25861159
  4. Abcd2 is a strong modifier of the metabolic impairments in peritoneal macrophages of ABCD1-deficient mice PMID:25255441
  5. Data indicate that astrocytes from adrenoleukodystrophy protein Abcd1-/- mice respond sensitively to long-term very-long-chain fatty acids (VLCFA) treatment. PMID:25583114
  6. Our data support a link between oxidative stress and the deficiency of Abcd1 or Acox1 peroxisomal proteins. PMID:22521832
  7. Study demonstrates that oxidative damage to proteins specifically affects five key enzymes of glycolysis and TCA (Tricarboxylic acid) cycle in spinal cords of Abcd1(-) mice. PMID:21453200
  8. ALDP facilitates the interaction between peroxisomes and mitochondria, resulting, when ALDP is deficient in X-ALD, in increased VLCFA accumulation PMID:12509471
  9. characterization of the quaternary structure and identification as a homomeric protein PMID:15276650
  10. Accumulation of very long-chain fatty acids does not affect mitochindrial function in Abcd1 protein deficiency. PMID:15772093
  11. Abcd1 and Abcd2 gene silencing sensitizes astrocytes for inflammation and may have a role in X-adrenoleukodystrophy PMID:18723473

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Subcellular Location
Peroxisome membrane; Multi-pass membrane protein. Mitochondrion membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein. Endoplasmic reticulum membrane; Multi-pass membrane protein.
Protein Families
ABC transporter superfamily, ABCD family, Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily
Tissue Specificity
Widely expressed at low levels with higher levels in heart, lung, intestine and spleen than in skeletal muscle, brain, liver and kidney.
Database Links

UNIGENE: Mm.365

KEGG: mmu:11666

STRING: 10090.ENSMUSP00000002084

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