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

in vitro E.coli expression system

中文名稱：

Recombinant Mouse ATP-binding cassette sub-family D member 1(Abcd1)
貨號：

CSB-CF001068MO
說明書：

MSDS Datasheet
規(guī)格：
來源：

in vitro E.coli expression system
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

基因名：

Abcd1
Uniprot No.：

P48410
別名：

Abcd1; Ald; Aldgh; ATP-binding cassette sub-family D member 1; Adrenoleukodystrophy protein; ALDP
種屬：

Mus musculus (Mouse)
蛋白長度：

Full length protein
表達區(qū)域：

1-736
氨基酸序列

MPVLSTPRPSRVTTLKRTAVVLALTAYGVHKIYPLVRQCLTPARGPQVPAGEPTQEASGA TATKAGMNRVFLQRLLALLRLLFPRVLCRETGLLALHSAALVSRTFLSVYVARLDGRLAR CIVRKDPRAFSWQLLQWLLIALPATFINSAIRYLEGQLALSFRSRLVAHAYGLYFSQQTY YRVSNMDGRLRNPDQSLTEDVVAFAASVAHLYSNLTKPLLDVAVTSYTLLRAARSRGAGT AWPSAIAGLVVFLTANVLRAFSPKFGELVAEEARRKGELRYMHSRVVANSEEIAFYGGHE VELALLQHSYQDLASQINLILLERLWYVMLEQFLMKYVWSASGLLMVAVPIITATGYAES DSEAMKKAALEMKEEELVSERTEAFTIARNLLTAAADATERIMSSYKEVTELAGYTARVY EMFQVFEDVKHCRFKRTGDLEEAQAGPGVMVQSGVHVEGPLKIQGQVVDVEQGIICENIP IITPTGEVVVASLNIRVEEGMHLLITGPNGCGKSSLFRILGGLWPTYSGVLYKPPPQRMF YIPQRPYMSVGSLRDQVIYPDSAEDMRRKGCSEQQLEAILGIVHLRHILQREGGWEAVCD WKDVLSGGEKQRIGMARMFYHRPKYALLDECTSAVSIDVEGKIFQAAKDAGIALLSITHR PSLWKYHTHLLQFDGEGGWKFEKLDSAARLSLTEEKQRLEQQLAGIPKMQGRLQELRQIL GEAAAPVQPLVPGVPT
Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
蛋白標簽：

N-terminal 10xHis-tagged
產品提供形式：

Liquid or Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
緩沖液：

Lyophilized from Tris/PBS-based buffer, 6% Trehalose, pH 8.0
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

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.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet & COA：

Please contact us to get it.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
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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亞細胞定位：

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.
蛋白家族：

ABC transporter superfamily, ABCD family, Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily
組織特異性：

Widely expressed at low levels with higher levels in heart, lung, intestine and spleen than in skeletal muscle, brain, liver and kidney.
數(shù)據庫鏈接：

KEGG: mmu:11666

STRING: 10090.ENSMUSP00000002084

UniGene: Mm.365