HMGCL Antibody

Datasheet
Code CSB-PA250156
Former Code(s) CSB-PA989267
Size US$299
Uniprot No. P35914
Image
  • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA250156(HMGCL Antibody) at dilution 1/20, on the right is treated with fusion protein. (Original magnification: ×200)
  • The image on the left is immunohistochemistry of paraffin-embedded Human prostate cancer tissue using CSB-PA250156(HMGCL Antibody) at dilution 1/20, on the right is treated with fusion protein. (Original magnification: ×200)
  • Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-3: SKOV3 cells, mouse heart tissue, Mouse brain tissue, Primary antibody: CSB-PA250156(HMGCL Antibody) at dilution 1/150, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 5 seconds
Immunogen Fusion protein of Human HMGCL
Raised in Rabbit
Species Reactivity Human,Mouse,Rat
Tested Applications ELISA,WB,IHC;ELISA:1:1000-1:2000,WB:1:200-1:1000,IHC:1:50-1:200
Relevance The protein encoded by this gene belongs to the HMG-CoA lyase family. It is a mitochondrial enzyme that catalyzes the final step of leucine degradation and plays a key role in ketone body formation. Mutations in this gene are associated with HMG-CoA lyase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
Form Liquid
Conjugate Non-conjugated
Storage Buffer -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
Purification Method Antigen affinity purification
Isotype IgG
Alias 3-hydroxymethyl-3-methylglutaryl-CoA lyase
Immunogen Species Homo sapiens (Human)
Protocols ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Target Names HMGCL
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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Function Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism.
Involvement in disease 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD)
Subcellular Location Mitochondrion matrix, Peroxisome
Protein Families HMG-CoA lyase family
Tissue Specificity Highest expression in liver. Expressed in pancreas, kidney, intestine, testis, fibroblasts and lymphoblasts. Very low expression in brain and skeletal muscle. The relative expression of isoform 2 (at mRNA level) is highest in heart (30%), skeletal muscle
Database Links

HGNC: 5005

OMIM: 246450

KEGG: hsa:3155

STRING: 9606.ENSP00000363614

UniGene: Hs.533444

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