P0090

Anti-PMP70−Atto 488 antibody produced in rabbit

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

• Synonyme(s) : Anti-70 kDa Peroxisonal membrane protein, Anti-ABCD3, Anti-PXMP1
• Numéro MDL: MFCD01865555
• Code UNSPSC : 12352203
• Nomenclature NACRES : NA.41

Propriétés

• Source biologique: rabbit
• Conjugué: Atto 488 conjugate
• Forme d'anticorps: affinity isolated antibody
• Type de produit anticorps: primary antibodies
• Clone: polyclonal
• Forme: buffered aqueous solution
• Espèces réactives: rat, mouse, human
• Conditionnement: antibody small pack of 25 μL
• Conditions de stockage: protect from light
• Concentration: 1.5-3 mg/mL
• Technique(s): direct immunofluorescence: 10-20 μg/mL using human HeLa cells
• Numéro d'accès UniProt: P28288
• Conditions d'expédition: dry ice
• Température de stockage: −20°C
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... ABCD3(5825) ; mouse ... Abcd3(19299) ; rat ... Abcd3(25270)

Description

Description générale

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.

Immunogène

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.

Actions biochimiques/physiologiques

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.

Forme physique

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

Clause de non-responsabilité

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.

Informations sur la sécurité

• Code de la classe de stockage: 12 - Non Combustible Liquids
• Point d'éclair (°F): Not applicable
• Point d'éclair (°C): Not applicable
• Équipement de protection individuelle: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)