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Key Documents

MAB2162

Sigma-Aldrich

Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4

ascites fluid, clone 1AL-2B4, Chemicon®

Sinónimos:

ALDP

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About This Item

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

biological source

mouse

Quality Level

antibody form

ascites fluid

antibody product type

primary antibodies

clone

1AL-2B4, monoclonal

species reactivity

human

manufacturer/tradename

Chemicon®

technique(s)

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

isotype

IgG1

NCBI accession no.

UniProt accession no.

shipped in

dry ice

target post-translational modification

unmodified

Gene Information

human ... ABCD1(215)

Specificity

Human adrenoleukodystrophy protein (ALDP). No cross-reactivity on mouse ALDP, mouse ALDRP and PMP 70. MAB2162 can be used in Western blot, immunofluorescence or immunohistochemical studies to analyze the ALD 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 mosaicism for the presence or absence of ALDP.

Immunogen

ALDP fragment from aa 495-648 as a fusion protein
Epitope: a.a. 495-648

Application

Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4 detects level of Adrenoleukodystrophy Protein & has been published & validated for use in ELISA, WB, IC, IH.
ELISA: 1:500-1:5,000

Western blot: 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

Physical form

Ascites. Liquid, does not contain any preservative.

Storage and Stability

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

Important Note: During shipment, small volumes of product will occasionally become entrapped in the seal of the product vial. For products with volumes of 200 μl or less, we recommend gently tapping the vial on a hard surface or briefly centrifuging the vial in a tabletop centrifuge to dislodge any liquid in the container′s cap.

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

10 - Combustible liquids

wgk_germany

nwg

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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A Novel Double Mutation in the ABCD1 Gene in a Patient with X-linked Adrenoleukodystrophy: Analysis of the Stability and Function of the Mutant ABCD1 Protein.
Morita, M; Kobayashi, J; Yamazaki, K; Kawaguchi, K; Honda, A; Sugai, K; Shimozawa et al.
JIMD Reports null
Miguel Agustin Contreras et al.
Archives of biochemistry and biophysics, 477(2), 211-218 (2008-07-08)
Krabbe disease is a neuroinflammatory disorder in which galactosylsphingosine (psychosine) accumulates in nervous tissue. To gain insight into whether the psychosine-induced effects in nervous tissue extend to peripheral organs, we investigated the expression of cytokines and their effects on peroxisomal
Masashi Morita et al.
Biological & pharmaceutical bulletin, 35(11), 1972-1979 (2012-11-06)
Very long chain fatty acid (VLCFA) metabolism in astrocytes is important for the maintenance of myelin structure in central nervous system. To analyze the contribution of the ABCD1-dependent and -independent pathways to VLCFA metabolism in astrocytes, we prepared human glioblastoma
Masashi Morita et al.
JIMD reports, 44, 23-31 (2018-06-22)
Mutations in the ABCD1 gene that encodes peroxisomal ABCD1 protein cause X-linked adrenoleukodystrophy (X-ALD), a rare neurodegenerative disorder. More than 70% of the patient fibroblasts with this missense mutation display either a lack or reduction of the ABCD1 protein because
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)

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