ABCD4 Antibody
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中文名稱:ABCD4兔多克隆抗體
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貨號:CSB-PA970746
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規(guī)格:¥2024
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圖片:
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其他:
產(chǎn)品詳情
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產(chǎn)品名稱:Rabbit anti-Homo sapiens (Human) ABCD4 Polyclonal antibody
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Uniprot No.:
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基因名:ABCD4
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別名:ABCD4; PXMP1L; Lysosomal cobalamin transporter ABCD4; ATP-binding cassette sub-family D member 4; PMP70-related protein; P70R; Peroxisomal membrane protein 1-like; PXMP1-L; Peroxisomal membrane protein 69; PMP69
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宿主:Rabbit
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反應(yīng)種屬:Human
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免疫原:Synthesized peptide derived from internal of Human ABCD4.
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免疫原種屬:Homo sapiens (Human)
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克隆類型:Polyclonal
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純化方式:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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濃度:It differs from different batches. Please contact us to confirm it.
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產(chǎn)品提供形式:Liquid
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應(yīng)用范圍:ELISA,WB
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推薦稀釋比:
Application Recommended Dilution WB 1:500-1:3000 -
Protocols:
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儲存條件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期:Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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功能:Lysosomal membrane protein that transports cobalamin (Vitamin B12) from the lysosomal lumen to the cytosol in an ATP-dependent manner. Targeted by LMBRD1 lysosomal chaperone from the endoplasmic reticulum to the lysosomal membrane. Then forms a complex with lysosomal chaperone LMBRD1 and cytosolic MMACHC to transport cobalamin across the lysosomal membrane.
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基因功能參考文獻(xiàn):
- endogenous ABCD4 was localized to both lysosomes and the ER, and its lysosomal localization was disturbed by knockout of LMBRD1 PMID: 27456980
- Data suggest that ABCD4 lysosomal targeting depends on co-expression of and interaction with LMBRD1; mutations in LMBRD1 and ABCD4 that result in cobalamin metabolism disorders cblF and cblJ (or mutations in ATPase domain) disrupt interactions between LMBRD1 and ABCD4. (LMBRD1 = nuclear export signal-interacting protein; ABCD4 = ATP-binding cassette, sub-family D (ALD), member 4) PMID: 28572511
- Results propose a model whereby membrane-bound LMBD1 and ABCD4 facilitate the vectorial delivery of lysosomal vitamin B12 to cytoplasmic MMACHC. PMID: 25535791
- mutations altering the putative ATPase domain of ABCD4 affect its function, suggesting that the ATPase activity of ABCD4 may be involved in intracellular processing of vitamin B12. PMID: 22922874
- Defects in ABCB4 have been found to cause progressive familial intrahepatic cholestasis type 3. PMID: 21514256
- expression tends to be correlated with the severity of X-linked adrenoleukodystrophy PMID: 15800013
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相關(guān)疾病:Methylmalonic aciduria and homocystinuria type cblJ (MAHCJ)
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亞細(xì)胞定位:Endoplasmic reticulum membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.
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蛋白家族:ABC transporter superfamily, ABCD family, Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily
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組織特異性:Ubiquitous.
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數(shù)據(jù)庫鏈接:
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