ACADS Antibody

說明書

中文名稱：

ACADS兔多克隆抗體
貨號：

CSB-PA001127ESR2HU
規格：

￥440
圖片：
Western blot
All lanes: Short-chain specific acyl-CoA dehydrogenase, mitochondrial antibody at 7μg/ml
Lane 1: 293T whole cell lysate
Lane 2: HepG2 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 44 kDa
Observed band size: 44 kDa

Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA001127ESR2HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human pancreatic tissue using CSB-PA001127ESR2HU at dilution of 1:100
其他：

產品詳情
產品評價
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靶點詳情

產品詳情

產品名稱：

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

P16219
基因名：

ACADS
別名：

ACAD3 antibody; ACADS antibody; ACADS_HUMAN antibody; Acyl Coenzyme A dehydrogenase; C2 to C3 short chain antibody; Acyl-CoA dehydrogenase; C2 to C3 short chain antibody; Acyl-CoA dehydrogenase; short chain antibody; Acyl-Coenzyme A dehydrogenase; short chain antibody; AI196007 antibody; Bcd-1 antibody; Bcd1 antibody; Butyryl CoA dehydrogenase antibody; Butyryl-CoA dehydrogenase antibody; EC 1.3.99.2 antibody; mitochondrial antibody; SCAD antibody; Short chain acyl CoA dehydrogenase antibody; Short-chain specific acyl-CoA dehydrogenase antibody; Short-chain specific acyl-CoA dehydrogenase; mitochondrial antibody; Unsaturated acyl CoA reductase antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial protein (1-260AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

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

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:1000
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.

產品評價

靶點詳情

功能：

Short-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, short-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 4 to 6 carbons long primary chains.
基因功能參考文獻：
1. a link between ACADS susceptibility variants and abnormal beta-oxidation consistent with known altered kinetics of these variants PMID: 28532786
2. Korean patients with Short-chain acyl-CoA dehydrogenase deficiency showed heterogenous clinical features and ACADS genotype. PMID: 27466294
3. Thirteen proteins had significant alteration in protein levels in patients carrying variation c.319C>T in ACADS compared to controls and they belonged to various pathways, such as the antioxidant system and amino acid metabolism. PMID: 24485985
4. Results show significant upregulation of LASP1 and SCAD protein levels in acute psychotic bipolar disorder samples. PMID: 24554194
5. IVD mutations in Asian populations are distinct from these in Western populations. PMID: 22004070
6. physiological concentrations of flavin adenine dinucleotide resulted in a spectacular enhancement of the thermal stabilities of SCAD and prevented enzymatic activity loss PMID: 21968293
7. In glucose-tolerant individuals the minor C-allele of rs2014355 of ACADS was associated with reduced measures of glucose-stimulated insulin release during an oral glucose tolerance test. PMID: 21211036
8. We therefore propose that SCAD misfolding leads to production of ROS, which in turn leads to fission and a grain-like structure of the mitochondrial reticulum. This finding indicates a toxic response elicited by misfolded p.Arg83Cys SCAD proteins PMID: 20371198
9. Molecular pathogenesis of a novel mutation, G108D, in ACADS identified in subjects with ACADS deficiency. PMID: 20376488
10. Reduction of catalytic activity and stability in polymorphic variant of SCAD (Gly185Ser) is caused by decreased flexibility in the tertiary conformation of the mutant enzyme. PMID: 12220177
11. Replacement of the catalytic glutamate in either short-chain acyl-CoA dehydrogenase (SCAD) or isovaleryl-CoA dehydrogenase (IVD)with glycine resulted in a several-fold reduction in affinity for substrate. PMID: 16376132
12. One of 220 SIDs cases was homozygous for the prevalent MCAD A985G mutation. PMID: 18045290
13. The c.319C>T mutation can lead to wide clinical and biochemical phenotypic variability, suggesting a complex multifactorial/polygenic condition. This should be screened for in individuals with multicore myopathy, particularly among the Ashkenazim. PMID: 18054510
14. A homozygous variant allele of the SCAD gene, 625G>A, was detected in new case of short-chain acyl-CoA dehydrogenase deficiency. PMID: 18175080
15. Extensive laboratory investigations indicate that the short-chain acyl-CoA dehydrogenase gene variant is likely preventing or delaying the normal expression of the Prader-Willi syndrome phenotype. PMID: 18184946
16. SCAD deficiency should be considered as a disorder of protein folding that can lead to clinical disease in combination with other genetic and environmental factors. PMID: 18523805
17. A deoxyribonucleic analysis revealed the presence of a 625G>A (G-to-A substitution at nucleotide 625) variant short-chain acyl-coenzyme A dehydrogenase gene polymorphism. PMID: 18539996
18. SCAD deficiency cause a disorder that leads to the accumulation of butyrylcarnitine and ethylmalonic acid in blood and urine. PMID: 18977676
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相關疾病：

Acyl-CoA dehydrogenase short-chain deficiency (ACADSD)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

Acyl-CoA dehydrogenase family
數據庫鏈接：

HGNC: 90

OMIM: 201470

KEGG: hsa:35

STRING: 9606.ENSP00000242592

UniGene: Hs.507076

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