ACADSB Antibody

中文名稱：

ACADSB兔多克隆抗體
貨號：

CSB-PA001128LA01HU
規(guī)格：

￥440
圖片：
Western Blot
Positive WB detected in: HEK293 whole cell lysate, HepG2 whole cell lysate, Rat liver tissue
All lanes: ACADSB antibody at 3µg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 48, 37 kDa
Observed band size: 48 kDa

Immunohistochemistry of paraffin-embedded human lung cancer using CSB-PA001128LA01HU at dilution of 1:100
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) ACADSB Polyclonal antibody
Uniprot No.：

P45954
基因名：

ACADSB
別名：

2 MEBCAD antibody; 2 methyl branched chain acyl CoA dehydrogenase antibody; 2 methylbutyryl CoA dehydrogenase antibody; 2 methylbutyryl coenzyme A dehydrogenase antibody; 2-MEBCAD antibody; 2-methyl branched chain acyl-CoA dehydrogenase antibody; 2-methylbutyryl-CoA dehydrogenase antibody; 2-methylbutyryl-coenzyme A dehydrogenase antibody; ACAD7 antibody; ACADSB antibody; ACDSB_HUMAN antibody; acyl CoA dehydrogenase; short/branched chain antibody; acyl Coenzyme A dehydrogenase short branched chain antibody; mitochondrial antibody; OTTHUMP00000020685 antibody; OTTHUMP00000046795 antibody; SBCAD antibody; Short/branched chain specific acyl-CoA dehydrogenase antibody; short/branched chain specific acyl-CoA dehydrogenase; mitochondrial antibody
宿主：

Rabbit
反應種屬：

Human, Rat
免疫原：

Recombinant Human Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial protein (34-253AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產(chǎn)品，ACADSB Antibody (CSB-PA001128LA01HU)，的標記方式是Non-conjugated。對于ACADSB Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產(chǎn)品名稱	應用
HRP	CSB-PA001128LB01HU	ACADSB Antibody, HRP conjugated	ELISA
FITC	CSB-PA001128LC01HU	ACADSB Antibody, FITC conjugated
Biotin	CSB-PA001128LD01HU	ACADSB 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(chǎn)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.

產(chǎn)品評價

靶點詳情

功能：

Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the removal of one hydrogen from C-2 and C-3 of the fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, acts specifically on short and branched chain acyl-CoA derivatives such as (S)-2-methylbutyryl-CoA as well as short straight chain acyl-CoAs such as butyryl-CoA. Plays an important role in the metabolism of L-isoleucine by catalyzing the dehydrogenation of 2-methylbutyryl-CoA, one of the steps of the L-isoleucine catabolic pathway. Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug.
基因功能參考文獻：
1. the c.1165 A>G mutation before knowing whether the optimal screening cut-off would minimize true positives or false negatives for SBCADD associated with this mutation. PMID: 23712021
2. Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients PMID: 22277967
3. These findings confirm that SBCAD deficiency can be identified through newborn screening by acylcarnitine analysis. PMID: 20547083
4. Differences between the rat and human enzyme at positions 383, 222, and 220 alter substrate specificity without affecting substrate binding. PMID: 12855692
5. Identification and characterization of an IVS3+3A>G mutation (c.303+3A>G) in the SBCAD gene, and provide evidence that this mutation is disease-causing. PMID: 16317551
6. Results examine the mechanistic basis for dysfunction of the common variant short-chain acyl-CoA dehydrogenases and demonstrate that mutations can have a large impact on the redox properties of the enzyme. PMID: 16331963
7. Results indicate that substrate redox activation occurs in short-chain acyl-CoA dehydrogenase leading to a large enzyme midpoint potential shift. PMID: 16331964
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相關(guān)疾病：

Short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

Acyl-CoA dehydrogenase family
組織特異性：

Ubiquitously expressed.
數(shù)據(jù)庫鏈接：

HGNC: 91

OMIM: 600301

KEGG: hsa:36

STRING: 9606.ENSP00000357873

UniGene: Hs.81934

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