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Key Documents

MAB2164

Sigma-Aldrich

Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6

ascites fluid, clone 2AL-1D6, Chemicon®

Synonym(s):

ALDP

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About This Item

UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41

biological source

mouse

Quality Level

100

conjugate

unconjugated

antibody form

ascites fluid

antibody product type

primary antibodies

clone

2AL-1D6, monoclonal

species reactivity

human

manufacturer/tradename

Chemicon®

technique(s)

ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry: suitable
western blot: suitable

isotype

IgG1

NCBI accession no.

NM_000033.2

UniProt accession no.

P33897

shipped in

dry ice

target post-translational modification

unmodified

Gene Information

human ... ABCD1(215)

Immunogen

ALDP1 fragment from aa 279 to 482 as a fusion protein
Epitope: a.a. 279-482

Application

Detect Adrenoleukodystrophy Protein using this Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 validated for use in ELISA, WB, IC, IH.
ELISA: 1:500-1:5,000

Immunoblotting: 1:500-1:5,000

Immunohistochemistry: 1:500-1:5,000

Immunocytochemistry: 1:500-1:5,000

Optimal working dilutions must be determined by the end user.
Research Category
Neuroscience
Research Sub Category
Neurodegenerative Diseases

Biochem/physiol Actions

Human ALDP. No cross reactivity with mouse ALDRP, PMP70. Cross reactivity to an unidentified 80 kD MW protein is present in lymphoblastoid cells, but not in several other cells or tissues tested. MAB2164 can be used to analyze ALDP protein in cells and tissues from normal individuals, or from patients with adrenoleukodystrophy (ALD) or Zellweger syndrome and related peroxisomal diseases, and provides an excellent marker for peroxisomes (Mosser et al. 1994). About 70% of ALD male patients have no detectable ALDP in fibroblasts or leukocytes. A similar proportion of carrier females may show mosaïcism for the presence or absence of ALDP.

Physical form

Ascites. Liquid, does not contain any preservative.

Preparation Note

Maintain at -20°C in undiluted aliquots up to 12 months. Avoid repeated freeze/thaw cycles.

Legal Information

CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany

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 Code

10 - Combustible liquids

WGK

nwg

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Hematopoietic stem-cell gene therapy is associated with restored white matter microvascular function in cerebral adrenoleukodystrophy.
Lauer, et al.
Nature Communications, 14, 1900-1900 (2023)
Stefan Schönberger et al.
Archives of neurology, 64(5), 651-657 (2007-03-14)
X-linked adrenoleukodystrophy (X-ALD) is the most common inherited peroxisomal disorder. It is caused by impaired function of ALD protein that results in accumulation of very long-chain fatty acids in tissues and body fluids. So far, hematopoietic stem cell transplantation (HSCT)

Global Trade Item Number

SKUGTIN
MAB216404053252578366

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