ACAD8 Antibody

文獻引用 (1) 說明書

產品詳情

產品名稱：

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
反應種屬：

Human, Mouse
免疫原：

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

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，ACAD8 Antibody (CSB-PA892457LA01HU)，的標記方式是Non-conjugated。對于ACAD8 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA892457LB01HU	ACAD8 Antibody, HRP conjugated	ELISA
FITC	CSB-PA892457LC01HU	ACAD8 Antibody, FITC conjugated
Biotin	CSB-PA892457LD01HU	ACAD8 Antibody, Biotin conjugated	ELISA

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
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:20-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) 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.

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.
數據庫鏈接：

HGNC: 87

OMIM: 604773

KEGG: hsa:27034

STRING: 9606.ENSP00000281182

UniGene: Hs.14791

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