过氧化物酶膜蛋白1抗体-抗体-抗体-生物在线
北京索莱宝科技有限公司
过氧化物酶膜蛋白1抗体

过氧化物酶膜蛋白1抗体

商家询价

产品名称: 过氧化物酶膜蛋白1抗体

英文名称: PXMP1/PMP70

产品编号: bs-8584R

产品价格: null

产品产地: 北京

品牌商标: BIOSS

更新时间: 2024-09-03T09:28:34

使用范围: WB=1:100-500,ELISA=1:500-1000,IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500,

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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.