P0090

Anti-PMP70−Atto 488 in Kaninchen hergestellte Antikörper

1.5-3 mg/mL, affinity isolated antibody, buffered aqueous solution

• Synonym(e): Anti-70 kDa Peroxisonal membrane protein, Anti-ABCD3, Anti-PXMP1
• UNSPSC Code: 12352203
• NACRES: NA.41
• MDL number: MFCD01865555
• Conjugate: Atto 488 conjugate
• Clone: polyclonal
• Application: IF (d)
• Citations: 11

Eigenschaften

• biological source: rabbit
• conjugate: Atto 488 conjugate
• antibody form: affinity isolated antibody
• antibody product type: primary antibodies
• clone: polyclonal
• form: buffered aqueous solution
• species reactivity: rat, mouse, human
• packaging: antibody small pack of 25 μL
• storage condition: protect from light
• concentration: 1.5-3 mg/mL
• technique(s): direct immunofluorescence: 10-20 μg/mL using human HeLa cells
• UniProt accession no.: P28288
• shipped in: dry ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... ABCD3(5825) ; mouse ... Abcd3(19299) ; rat ... Abcd3(25270)

Beschreibung

General description

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.

Immunogen

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.

Application

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

Biochem/physiol Actions

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.

Physical form

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Sicherheitshinweise

• Lagerklasse: 12 - Non Combustible Liquids
• flash_point_f: Not applicable
• flash_point_c: Not applicable
• ppe: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)