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  4. Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial (ACADS)

Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial (ACADS)

  • 中文名稱：
    Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADS)
  • 貨號：
    CSB-YP001127HU
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADS)
  • 貨號：
    CSB-EP001127HU
  • 規格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADS)
  • 貨號：
    CSB-EP001127HU-B
  • 規格：
  • 來源：
    E.coli
  • 共軛：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名稱：
    Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADS)
  • 貨號：
    CSB-BP001127HU
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADS)
  • 貨號：
    CSB-MP001127HU
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    ACADS
  • Uniprot No.：
  • 別名：
    ACAD3; ACADS; ACADS_HUMAN; Acyl Coenzyme A dehydrogenase; C2 to C3 short chain; Acyl-CoA dehydrogenase; C2 to C3 short chain; Acyl-CoA dehydrogenase; short chain; Acyl-Coenzyme A dehydrogenase; short chain; AI196007; Bcd-1; Bcd1; Butyryl CoA dehydrogenase; Butyryl-CoA dehydrogenase; EC 1.3.99.2; mitochondrial; SCAD; Short chain acyl CoA dehydrogenase; Short-chain specific acyl-CoA dehydrogenase; Short-chain specific acyl-CoA dehydrogenase; mitochondrial; Unsaturated acyl CoA reductase
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length of Mature Protein
  • 表達區域：
    25-412
  • 氨基酸序列
    LHTIYQ SVELPETHQM LLQTCRDFAE KELFPIAAQV DKEHLFPAAQ VKKMGGLGLL AMDVPEELGG AGLDYLAYAI AMEEISRGCA STGVIMSVNN SLYLGPILKF GSKEQKQAWV TPFTSGDKIG CFALSEPGNG SDAGAASTTA RAEGDSWVLN GTKAWITNAW EASAAVVFAS TDRALQNKGI SAFLVPMPTP GLTLGKKEDK LGIRGSSTAN LIFEDCRIPK DSILGEPGMG FKIAMQTLDM GRIGIASQAL GIAQTALDCA VNYAENRMAF GAPLTKLQVI QFKLADMALA LESARLLTWR AAMLKDNKKP FIKEAAMAKL AASEAATAIS HQAIQILGGM GYVTEMPAER HYRDARITEI YEGTSEIQRL VIAGHLLRSY RS
  • 蛋白標簽：
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

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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