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MAB2164

Sigma-Aldrich

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

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

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别名:
ALDP
UNSPSC代码:
12352203
eCl@ss:
32160702
NACRES:
NA.41

生物来源

mouse

质量水平

抗体形式

ascites fluid

抗体产品类型

primary antibodies

克隆

2AL-1D6, monoclonal

种属反应性

human

制造商/商品名称

Chemicon®

技术

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

同位素/亚型

IgG1

NCBI登记号

UniProt登记号

运输

dry ice

靶向翻译后修饰

unmodified

基因信息

human ... ABCD1(215)

特异性

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.

免疫原

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

应用

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

外形

Ascites. Liquid, does not contain any preservative.

储存及稳定性

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

法律信息

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

免责声明

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.

WGK

nwg

闪点(°F)

Not applicable

闪点(°C)

Not applicable


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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)

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