Anti-PMP70−Atto 488 兔抗

70 kDa peroxisomal membrane protein (PMP70) belongs to the adrenoleukodystrophy (ALD) subfamily of the ATP-binding cassette (ABC) transporter superfamily. It is a half-size ABC integral membrane protein consisting of 6 transmembrane domains and one ATP-binding domain.

ATP-binding cassette sub-family D member 3 (ABCD3) is a peroxisomal membrane protein belonging to an ATP binding cassette family. The gene encoding it is localized on human chromosome 1p21-22 and rat chromosome 2q41.

synthetic peptide corresponding to amino acid residues 644-659 of rat PMP70 conjugated to KLH. The corresponding sequence is identical in mouse and differs by one amino acid in human.

Atto 488 antibody produced in rabbit has been used in immunofluorescence.

70 kDa peroxisomal membrane protein (PMP70) forms a stable complex with the adrenoleukodystrophy protein, (ALDP), and several other peroxisomal proteins. ATP-binding/hydrolysis by PMP70 and ALDL and their phosphorylation are involved in the regulation of fatty acid transport into peroxisomes. Mutations in the PMP70 (PXMP1) gene may cause a subset of Zellweger syndrome-2, an autosomal recessive disorder that is manifested by a defective import mechanisms for peroxisomal matrix enzymes.

ATP-binding cassette sub-family D member 3 (ABCD3) is responsible for the transport of fatty acids into peroxisomes by an ATP-dependent mechanism. It also participates in the oxidation of dicarboxylic acids. Defects in the protein activity result in hepatosplenomegaly, a liver disease. Change in the expression of ABCD3 is associated with prostate tumor aggressiveness and a deficiency of the protein causes bile acid biosynthesis defect.

0.01M 磷酸缓冲盐溶液，pH 7.4，含 15mM 叠氮化钠。

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