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ACADS Antibody

  • 货号：
    CSB-PA001127GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P16219
  • 基因名：
    ACADS
  • 别名：
    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
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human ACADS
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Acyl-CoA dehydrogenase short-chain deficiency (ACADSD)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Acyl-CoA dehydrogenase family
  • 数据库链接：

    HGNC: 90

    OMIM: 201470

    KEGG: hsa:35

    STRING: 9606.ENSP00000242592

    UniGene: Hs.507076