推荐产品
生物源
mouse
品質等級
抗體表格
ascites fluid
抗體產品種類
primary antibodies
無性繁殖
2AL-1D6, monoclonal
物種活性
human
製造商/商標名
Chemicon®
技術
ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry: suitable
western blot: suitable
同型
IgG1
NCBI登錄號
UniProt登錄號
運輸包裝
dry ice
目標翻譯後修改
unmodified
基因資訊
human ... ABCD1(215)
特異性
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
應用
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.
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
Neuroscience
Research Sub Category
Neurodegenerative Diseases
Neurodegenerative Diseases
外觀
Ascites. Liquid, does not contain any preservative.
儲存和穩定性
Maintain at -20°C in undiluted aliquots up to 12 months. Avoid repeated freeze/thaw cycles.
法律資訊
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
免責聲明
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.
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儲存類別代碼
10 - Combustible liquids
水污染物質分類(WGK)
nwg
閃點(°F)
Not applicable
閃點(°C)
Not applicable
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
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
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)
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.
Annals of the New York Academy of Sciences, 804, 461-476 (1996)
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