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Merck

SAB4200181

Sigma-Aldrich

Anti-PMP70 antibody, Mouse monoclonal

enhanced validation

clone 70-18, purified from hybridoma cell culture

Sinónimos:

Anti- ZWS2; ZELLWEGER SYNDROME 2, Anti-70 kDa Peroxisomal membrane Protein, Anti-ABCD3, Anti-ATP-binding cassette, sub-family D (ALD), member 3, Anti-PXMP1

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About This Item

MDL number:
UNSPSC Code:
12352203
NACRES:
NA.41

biological source

mouse

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

70-18, monoclonal

form

buffered aqueous solution

mol wt

antigen ~70 kDa

species reactivity

rat, mouse, human

packaging

antibody small pack of 25 μL

enhanced validation

independent
Learn more about Antibody Enhanced Validation

concentration

~1.0 mg/mL

technique(s)

immunofluorescence: suitable
western blot: 1-2 μg/mL using whole extracts of human HepG2 or rat PC12 cells

isotype

IgG1

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... ABCD3(5825)
mouse ... Abcd3(19299)
rat ... Abcd3(25270)

General description

Anti-PMP70 antibody, Mouse monoclonal (mouse IgG1 isotype) is derived from the hybridoma 70-18 produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice. The 70 kDa peroxisomal membrane protein (PMP70), also designated PXMP1 and ABCD3 or ABD3, is one of the major components of peroxisomal membranes. PMP70 belongs to the 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.

Application

Anti-PMP70 antibody, Mouse monoclonal has been used in immunoblotting and immunofluorescence.

Biochem/physiol Actions

The 70 kDa peroxisomal membrane protein (PMP70) participates in the metabolic transport of long and very long fatty acids into peroxisomes. It forms a stable complex with the adrenoleukodystrophy protein, adrenoleukodystrophy protein (ALDP), and several other peroxisomal proteins. Mutations in the PMP70 (PXMP1) gene may cause a subset of Zellweger syndrome-2, an autosomal recessive disorder that is manifested by defective import mechanisms for peroxisomal matrix enzymes.

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.

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Storage Class

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable


Certificados de análisis (COA)

Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»

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Visite la Librería de documentos

Kareem Soliman et al.
Scientific reports, 8(1), 7809-7809 (2018-05-19)
Peroxisomes are ubiquitous cell organelles involved in many metabolic and signaling functions. Their assembly requires peroxins, encoded by PEX genes. Mutations in PEX genes are the cause of Zellweger Syndrome spectrum (ZSS), a heterogeneous group of peroxisomal biogenesis disorders (PBD).
Characterization of the 70-kDa peroxisomal membrane protein, an ATP binding cassette transporter
Imanaka T, et al.
The Journal of Biological Chemistry, 274(17), 11968-11976 (1999)
A close relative of the adrenoleukodystrophy (ALD) gene codes for a peroxisomal protein with a specific expression pattern
Lombard-Platet G, et al.
Proceedings of the National Academy of Sciences of the USA, 93(3), 1265-1269 (1996)
Alaumy Joshi et al.
The Journal of biological chemistry, 293(44), 16953-16963 (2018-09-22)
Polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and cataract (PHARC) is a rare genetic human neurological disorder caused by null mutations to the Abhd12 gene, which encodes the integral membrane serine hydrolase enzyme ABHD12. Although the role that ABHD12 plays in
Nancy C Li et al.
PloS one, 11(2), e0149728-e0149728 (2016-02-24)
Sterol carrier protein-2 (SCP2), also called nonspecific lipid-transfer protein, is thought to play a major role in intracellular lipid transport and metabolism, and it has been associated with diseases involving abnormalities in lipid trafficking, such as Zellweger syndrome. The Scp2

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