产品名称
HMGCL (19P8) Rabbit Monoclonal Antibody
别名
HMG CoA lyase; HMGCL; Hydroxymethylglutaricaciduria; MS725;
纯度
Affinity-chromatography
存储缓冲液
Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% New type preservative N and 0.05% BSA.
Human Swissprot No.
P35914
免疫原
A synthetic peptide of human HMGCL
稀释度
WB: 1:1000 IHC: 1:100
注意事项
HMGCL Antibody is for research use only and not for use in diagnostic or therapeutic procedures.
组织表达
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 (22%), and brain (14%).
细胞定位
Mitochondrion matrix {ECO:0000250|UniProtKB:P38060}. Peroxisome {ECO:0000250|UniProtKB:P38060}. Note=Unprocessed form is peroxisomal {ECO:0000250|UniProtKB:P38060}
功能
Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3- methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. 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.
