Recombinant ABCD1 抗体
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北京 101111
Quick Overview for Recombinant ABCD1 抗体 (ABIN7880023)
抗原
See all ABCD1 抗体抗体类型
适用
宿主
克隆类型
标记
应用范围
克隆位点
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原理
- ABCD1 Antibody / ATP-binding cassette sub-family D member 1
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纯化方法
- Affinity-chromatography
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免疫原
- A synthesized peptide derived from human ABCD1 / ALD was used as the immunogen for the ABCD1 antibody.
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亚型
- IgG
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anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) (AA 257-285) antibody
ABCD1 适用: 人 WB, FACS, IHC (p) 宿主: 兔 Polyclonal RB18846 unconjugated
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) antibodyKD Validated ABCD1 适用: 人 WB 宿主: 兔 Monoclonal 23GB3125 unconjugated Recombinant Antibody
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) antibodyABCD1 适用: 人, 小鼠 ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) (AA 264-293), (Middle Region) antibodyABCD1 适用: 人 WB, IHC (p), EIA 宿主: 兔 Polyclonal unconjugated
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) antibodyABCD1 适用: 人, 小鼠, 大鼠 IF 宿主: 兔 Polyclonal unconjugated
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) (Internal Region) antibodyVerified ABCD1 适用: 人 WB, ELISA 宿主: 山羊 Polyclonal unconjugated
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) (AA 1-100) antibodyABCD1 适用: 人 WB, ELISA 宿主: 小鼠 Polyclonal unconjugated
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) (AA 1-745) antibodyABCD1 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) (AA 632-745) antibodyABCD1 适用: 人 ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1) (Internal Region) antibodyABCD1 适用: 人 WB, ELISA 宿主: 兔 Polyclonal unconjugated
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应用备注
- Optimal dilution of the ABCD1 antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- Rabbit IgG in phosphate buffered saline, pH 7.4, 150 mM NaCl, 0.02 % sodium azide and 50 % glycerol, 0.4-0.5 mg/mL BSA
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store the ABCD1 antibody at -20oC.
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- ABCD1 (ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1))
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别名
- ABCD1
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背景
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ABCD1 antibody detects ATP-binding cassette sub-family D member 1, a peroxisomal membrane transporter involved in the import of very-long-chain fatty acids (VLCFAs) into peroxisomes for degradation. ABCD1 belongs to the ABC transporter family and is encoded by a gene on the X chromosome. Loss-of-function mutations in ABCD1 cause X-linked adrenoleukodystrophy (X-ALD), a severe metabolic disorder characterized by accumulation of VLCFAs in plasma and tissues, leading to demyelination and adrenal insufficiency.
Research using ABCD1 antibody has demonstrated its critical role in peroxisomal lipid metabolism. ABCD1 works as a half-transporter that dimerizes, either with itself or with other family members such as ABCD2, to import VLCFAs across the peroxisomal membrane. Disruption of this transport pathway leads to impaired beta-oxidation and pathological lipid accumulation. In the nervous system, VLCFA buildup damages myelin and causes neuroinflammation, while in endocrine tissues it impairs adrenal steroidogenesis.
Clinical studies highlight ABCD1 as the causative gene in X-ALD, which manifests in childhood cerebral ALD, adrenomyeloneuropathy (AMN), and Addison-only disease. Mutations in ABCD1 result in variable phenotypes, even within the same family, suggesting additional genetic and environmental modifiers. Antibody-based detection of ABCD1 supports diagnostic studies and research into genotype-phenotype correlations.
In therapeutic research, ABCD1 is a target for gene therapy and stem cell transplantation, both of which aim to restore peroxisomal function and prevent neurological decline. Monitoring ABCD1 expression with antibodies enables evaluation of therapeutic efficacy. Studies also indicate compensatory roles for ABCD2 and ABCD3 transporters, making antibody-based tools essential for dissecting overlapping functions.
Antibodies against ABCD1 are validated for immunohistochemistry, western blot, and immunofluorescence. These reagents allow detection of peroxisomal localization, quantification of expression, and investigation of functional restoration in therapeutic models. By enabling precise measurement of ABCD1, they support basic, clinical, and translational research.
NSJ Bioreagents supplies this ABCD1 antibody for studies in peroxisomal metabolism, neurodegeneration, and rare genetic disorders. -
UniProt
- P33897
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途径
- Monocarboxylic Acid Catabolic Process
抗原
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