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MAB2164

Sigma-Aldrich

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

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

Sinónimos:

ALDP

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About This Item

Código UNSPSC:
12352203
eCl@ss:
32160702
NACRES:
NA.41
clon:
2AL-1D6, monoclonal
application:
ELISA
ICC
IHC
WB
reactividad de especies:
human
técnicas:
ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry: suitable
western blot: suitable
citations:
2

origen biológico

mouse

Nivel de calidad

forma del anticuerpo

ascites fluid

tipo de anticuerpo

primary antibodies

clon

2AL-1D6, monoclonal

reactividad de especies

human

fabricante / nombre comercial

Chemicon®

técnicas

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

isotipo

IgG1

Nº de acceso NCBI

Nº de acceso UniProt

Condiciones de envío

dry ice

modificación del objetivo postraduccional

unmodified

Información sobre el gen

human ... ABCD1(215)

Especificidad

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.

Inmunógeno

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

Aplicación

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

Forma física

Ascites. Liquid, does not contain any preservative.

Almacenamiento y estabilidad

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

Información legal

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

Cláusula de descargo de responsabilidad

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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Código de clase de almacenamiento

10 - Combustible liquids

Clase de riesgo para el agua (WGK)

nwg

Punto de inflamabilidad (°F)

Not applicable

Punto de inflamabilidad (°C)

Not applicable


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M Contreras et al.
Archives of biochemistry and biophysics, 334(2), 369-379 (1996-10-15)
Adrenoleukodystrophy (X-ALD) is a demyelinating disorder characterized by the accumulation of saturated very-long-chain fatty acids (> C22:0) due to the impaired activity of lignoceroyl-CoA ligase. The gene responsible for the disease was found to code for a 84-kDa peroxisomal integral
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
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)
J Mosser et al.
Human molecular genetics, 3(2), 265-271 (1994-02-01)
Adrenoleukodystrophy is a severe genetic demyelinating disease associated with an impairment of beta-oxidation of very long chain fatty acids (VLCFA) in peroxisomes. Earlier studies had suggested that a deficiency in VLCFA CoA synthetase was the primary defect. A candidate adrenoleukodystrophy
X-linked adrenoleukodystrophy.
Aubourg, P and Mandel, J L
Annals of the New York Academy of Sciences, 804, 461-476 (1996)

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