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ACADL Antibody (N-term) 精选

Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
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  • 1 - ACADL Antibody (N-term) AP13134a
    Western blot analysis of lysates from HepG2 cell line and mouse kidney, rat spleen tissue lysates(from left to right), using ACADL Antibody (N-term)(Cat. #AP13134a). AP13134a was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:5000 dilution was used as the secondary antibody. Lysates at 35ug per lane.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, E
Primary Accession P28330
Other Accession NP_001599.1
Reactivity Human, Rat, Mouse
Predicted Bovine, Canine, Rabbit
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 47656 Da
Antigen Region 14-43 aa
Additional Information
Gene ID 33
Other Names Long-chain specific acyl-CoA dehydrogenase, mitochondrial, LCAD, ACADL
Target/Specificity This ACADL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 14-43 amino acids from the N-terminal region of human ACADL.
Dilution WB~~1:1000
E~~Use at an assay dependent concentration.
Format Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsACADL Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name ACADL (HGNC:88)
Function Catalyzes the first of the four reactions of the mitochondrial fatty acid beta-oxidation (FAO) pathway, which consists in the proR-proR stereospecific alpha, beta-dehydrogenation of fatty acyl-CoA thioesters using the electron transfer flavoprotein (ETF) as their physiologic electron acceptor, resulting in the formation of trans-2-enoyl-CoA ((2E)-enoyl-CoA) (PubMed:17564966, PubMed:21237683, PubMed:24591516, PubMed:32389575). The mitochondrial FAO pathway is the major energy-producing process in tissues and is performed through cycles of four consecutive reactions (PubMed:17564966, PubMed:26474213). Each FAO cycle shortens the fatty acyl-CoA by two carbons, yielding one acetyl-CoA (for the citric acid cycle), one FADH(2), and one NADH (which donate electrons to the respiratory chain for ATP production) (PubMed:17564966, PubMed:26474213). Among the different mitochondrial acyl-CoA dehydrogenases, long-chain specific acyl-CoA dehydrogenase activity overlaps with that of ACADV and ACAD9, acting on saturated and unsaturated acyl-CoAs with 6 to 24 carbons with a preference for 8 to 18 carbons long primary chains (PubMed:17564966, PubMed:21237683, PubMed:32389575, PubMed:8823175). Can use (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate as substrate in vitro (which is not primarily used for energy but mainly beta-oxidized in the peroxisomes) (PubMed:17564966, PubMed:26474213). In addition, based on its established catalytic mechanism, and combined genetic interaction or mutant phenotype evidence, it is predicted to act also on substrates that have not been tested experimentally but are metabolized by mitochondrial FAO, including long-chain unsaturated fatty acids such as linoleate (9Z,12Z-octadecadienoate), linolenate (9Z,12Z,15Z- octadecatrienoate), and others (PubMed:26474213). Plays a primary role in FAO in tissues where it is the main long-chain ACAD expressed, such as the lung, specifically in type 2 alveolar cells (responsible for surfactant production) (PubMed:17564966, PubMed:24591516). Probably responsible for beta-oxidation of bulky substrates including branched chain fatty acyl-CoAs and sterol derivatives thanks to its enlarged substrate-binding cavity (PubMed:38839792).
Cellular Location Mitochondrion matrix {ECO:0000250|UniProtKB:P15650}
Tissue Location Expressed at mRNA and protein levels in lungs, where it localizes specifically in alveolar epithelial cells (alveolar type II pneumocytes) (PubMed:17564966, PubMed:24591516). Also expressed at mRNA levels in prostate, thyroid gland, kidney, heart and muscle (PubMed:17564966).
Research Areas

BACKGROUND

The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia.

REFERENCES

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Maher, A.C., et al. Mol. Genet. Metab. 100(2):163-167(2010)
Illig, T., et al. Nat. Genet. 42(2):137-141(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
Lu, Y., et al. J. Lipid Res. 49(12):2582-2589(2008)

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