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产品信息
PA5-30139
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Recombinant protein fragment corresponding to a region within amino acids 38 and 296 of Human ABCD4
偶联物
形式
Liquid
浓度
0.79 mg/mL
纯化类型
Antigen affinity chromatography
保存液
0.1M tris glycine, pH 7, with 20% glycerol
内含物
0.01% thimerosal
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
运输条件
Wet ice
RRID
AB_2547613
产品详细信息
Recommended positive controls: 293T, A431.
Predicted reactivity: Human (99%), Mouse (94%), Rat (94%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
靶标信息
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.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: 69 kDa peroxisomal ABC-transporter; ATP-binding cassette sub-family D member 4; ATP-binding cassette, sub-family D (ALD), member 4; Lysosomal cobalamin transporter ABCD4; P70R; Peroxisomal membrane protein 1-like; Peroxisomal membrane protein 69; PMP69; PMP70-related protein; PXMP1-L
基因别名: ABC41; ABCD4; EST352188; MAHCJ; P70R; P79R; PMP69; PXMP1L
UniProt ID: (Human) O14678
Entrez Gene ID: (Human) 5826
