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
1AL-2B4, 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 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.
免疫原
ALDP fragment from aa 495-648 as a fusion protein
Epitope: a.a. 495-648
應用
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.
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
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 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.
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.
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
The TRIM37 gene encodes a peroxisomal RING-B-box-coiled-coil protein: classification of mulibrey nanism as a new peroxisomal disorder.
American Journal of Human Genetics null
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.
JIMD Reports null
Adrenoleukodystrophy: subcellular localization and degradation of adrenoleukodystrophy protein (ALDP/ABCD1) with naturally occurring missense mutations.
Journal of Neurochemistry null
The Journal of biological chemistry, 277(32), 28765-28773 (2002-06-06)
X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder characterized by accumulation of very long-chain fatty acids (VLCFA). This accumulation has been attributed to decreased VLCFA beta-oxidation and peroxisomal very long-chain acyl-CoA synthetase (VLCS) activity. The X-ALD gene, ABCD1, encodes a peroxisomal
Genomic profiling identifies novel mutations and SNPs in ABCD1 gene: a molecular, biochemical and clinical analysis of X-ALD cases in India.
Testing null
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