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The protein encoded by HMGCL belongs to the HMG-CoA lyase family.
Showing 10 out of 111 products:
Human Monoclonal HMGCL Primary Antibody für ELISA, WB - ABIN561291
Puisac, Arnedo, Casale, Ribate, Castiella, Ramos, Ribes, Pérez-Cerdá, Casals, Hegardt, Pié: Differential HMG-CoA lyase expression in human tissues provides clues about 3-hydroxy-3-methylglutaric aciduria. in Journal of inherited metabolic disease 2010
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Human Polyclonal HMGCL Primary Antibody für WB - ABIN4319409
Youm, Nguyen, Grant, Goldberg, Bodogai, Kim, DAgostino, Planavsky, Lupfer, Kanneganti, Kang, Horvath, Fahmy, Crawford, Biragyn, Alnemri, Dixit: The ketone metabolite β-hydroxybutyrate blocks NLRP3 inflammasome-mediated inflammatory disease. in Nature medicine 2015
Human Polyclonal HMGCL Primary Antibody für IHC, IHC (p) - ABIN4319410
Martinez-Outschoorn, Lin, Whitaker-Menezes, Howell, Lisanti, Sotgia: Ketone bodies and two-compartment tumor metabolism: stromal ketone production fuels mitochondrial biogenesis in epithelial cancer cells. in Cell cycle (Georgetown, Tex.) 2012
Data suggest that HMGCS1 (HMG-CoA synthase (zeige HMGCS1 Antikörper) 1) signals through ketogenesis/acetoacetate to promote cell proliferation and BRAF (zeige BRAF Antikörper)(V600E)-dependent MEK1 (zeige MAP2K1 Antikörper) activation in BRAF (zeige BRAF Antikörper)(V600E)-positive melanoma and colon cancer cells; HMGCS1 (zeige HMGCS1 Antikörper) co-localizes with HMGCL (HMG-CoA lyase) and BRAF (zeige BRAF Antikörper)(V600E) in cytosol of melanoma and colon cancer cells. (BRAF (zeige BRAF Antikörper) = proto-oncogene (zeige RAB1A Antikörper) protein B (zeige LEPREL2 Antikörper)-raf (zeige SNRPE Antikörper))
The genetic analysis revealed a novel homozygote deletion in exon 3 and 4 in HMGCL gene. HMG-CoA lyase deficiency should be thought in the patients with hypoketotic hypoglycemia, hyperammonemia, elevated liver function tests, noncompaction left ventricle and characteristic white matter changes and in the differential diagnosis of macrocephaly.
this is the first study describing HMGCL deficiency caused by uniparental disomy.
This efficient UPLC-MS/MS assay permits rapid and high sensitive determination of HMGCR (zeige HMGCR Antikörper) enzyme activity, tracing potential alterations in cholesterol biosynthesis.
analysis of HMGCLL1 (zeige HMGCLL1 Antikörper) as an extramitochondrial human 3-hydroxy-3-methylglutaryl-CoA lyase and comparison with MHGCL
An alternative transcript of HMGCS2 (zeige HMGCS2 Antikörper) carrying a deletion of exon 4, and two alternative transcripts of HMGCL with deletions of exons 5 and 6, and exons 5, 6 and 7, respectively, were detected.
levels of enzyme activity do not strongly correlate with formation of inter-subunit adducts by HMGCL mutants. C170S, C266S, and C323S proteins do not form inter-subunit disulfide adducts but such an adduct is restored in the C170S/C174S double mutant.
Crystal structures of ternary complexes of WT HMGCL with the competitive inhibitor 3-hydroxyglutaryl-CoA and of the catalytically deficient HMGCL R41M mutant with substrate HMG-CoA have been determined to 2.4 and 2.2 A.
We report on a new case of 3-hydroxy-3-methylglutaric aciduria particular by its late onset in a 3-year-old patient. Molecular investigation identified two new sequence modifications in the HMGCL gene: c.494G>A (p.Arg165Gln) and c.820G>A (p.Gly274Arg).
A role is suggested for arginine-41 in deprotonation or enolization of acetyldithio-CoA, implicating this residue in the HMG (zeige SSRP1 Antikörper)-CoA cleavage reaction chemistry that leads to acetyl-CoA (zeige LPCAT2 Antikörper) product formation.
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.
3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase
, 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria)
, 3-hydroxy-3-methylglutarate-CoA lyase
, HMG-CoA lyase
, hydroxymethylglutaryl-CoA lyase, mitochondrial
, 3-hydroxy-3-methylglutaryl-CoA lyase
, mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase
, 3-hydroxy-3-methylglutaryl-Coenzyme A lyase