ACAD8 Antibody, HRP conjugated

說明書

中文名稱：

ACAD8兔多克隆抗體, HRP偶聯(lián)
貨號：

CSB-PA892457LB01HU
規(guī)格：

￥880
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) ACAD8 Polyclonal antibody
Uniprot No.：

Q9UKU7
基因名：

ACAD8
別名：

ACAD 8 antibody; ACAD-8 antibody; ACAD8 antibody; ACAD8_HUMAN antibody; Activator-recruited cofactor 42 kDa component antibody; Acyl CoA dehydrogenase family member 8 antibody; Acyl Coenzyme A dehydrogenase family member 8 antibody; Acyl-CoA dehydrogenase family member 8 antibody; ARC42 antibody; FLJ22590 antibody; IBD antibody; Isobutyryl-CoA dehydrogenase antibody; Isobutyryl-CoA dehydrogenase; mitochondrial antibody; mitochondrial antibody
宿主：

Rabbit
反應(yīng)種屬：

Human
免疫原：

Recombinant Human Isobutyryl-CoA dehydrogenase, mitochondrial protein (201-415AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

HRP
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA
Protocols：

ELISA Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

功能：

Isobutyryl-CoA dehydrogenase which catalyzes one of the steps of the valine catabolic pathway. To a lesser extent, is also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA.
基因功能參考文獻：
1. we discovered a novel c.1156_1158delCAG mutation in ACAD8 in patients with isobutyryl-CoA dehydrogenase deficiency , and investigated the mutation spectrum of ACAD8. PMID: 24635911
2. first enzymatic and molecular confirmation of a deficiency of this enzyme in a patient PMID: 12359132
相關(guān)疾病：

Isobutyryl-CoA dehydrogenase deficiency (IBDD)
亞細胞定位：

Mitochondrion.
蛋白家族：

Acyl-CoA dehydrogenase family
組織特異性：

Detected at comparable levels in all tissues examined (heart, lung, brain, skeletal muscle, pancreas and placenta). Weakly expressed in liver and kidney.
數(shù)據(jù)庫鏈接：

HGNC: 87

OMIM: 604773

KEGG: hsa:27034

STRING: 9606.ENSP00000281182

UniGene: Hs.14791

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