3-Hydroxymethyl-3-Methylglutaryl-CoA Lyase (HMGCL) Kits ELISA

The protein encoded by HMGCL belongs to the HMG-CoA lyase family. De plus, nous expédions HMGCL Anticorps (114) et HMGCL Protéines (16) et beaucoup plus de produits pour cette protéine.

list all ELISA KIts Gène GeneID UniProt
HMGCL 15356 P38060
HMGCL 3155 P35914
HMGCL 79238 P97519
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Top HMGCL Kits ELISA sur anticorps-enligne.fr

Showing 4 out of 7 products:

Catalogue No. Reactivité Sensibilité Gamme Images Quantité Livraison Prix Détails
Boeuf (Vache) 5.8 pg/mL 23.5-1500 pg/mL Typical standard curve 96 Tests 15 to 18 Days
Humain < 0.188 ng/mL 0.313 ng/mL - 20 ng/mL   96 Tests 11 to 18 Days
Souris < 46.9 pg/mL 78 pg/mL - 5000 pg/mL   96 Tests 11 to 18 Days
Rat < 0.094 ng/mL 0.156 ng/mL - 10 ng/mL   96 Tests 11 to 18 Days

Plus Kits ELISA pour HMGCL partenaires d'interaction

Human 3-Hydroxymethyl-3-Methylglutaryl-CoA Lyase (HMGCL) interaction partners

  1. The expression of HMGCL was silenced in nasopharyngeal carcinoma tissue. Downregulation of HMGCL in nasopharyngeal carcinoma was associated with low intracellular beta-hydroxybutyrate (beta-HB) production, thereby reducing reactive oxygen species (ROS) generation.

  2. Data suggest that HMGCS1 (HMG-CoA synthase 1) signals through ketogenesis/acetoacetate to promote cell proliferation and BRAF(V600E)-dependent MEK1 activation in BRAF(V600E)-positive melanoma and colon cancer cells; HMGCS1 co-localizes with HMGCL (HMG-CoA lyase) and BRAF(V600E) in cytosol of melanoma and colon cancer cells. (BRAF = proto-oncogene protein B-raf)

  3. 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.

  4. this is the first study describing HMGCL deficiency caused by uniparental disomy.

  5. This efficient UPLC-MS/MS assay permits rapid and high sensitive determination of HMGCR enzyme activity, tracing potential alterations in cholesterol biosynthesis.

  6. in the 2 stop codon mutations c.109G>T and c.504_505delCT studied, the stop codon does not appear to be the cause of aberrant splicing; the mutation c.504_505delCT causes 2 mRNA transcripts with a stop codon that generate two simultaneous nonsense-mediated mRNA decay phenomena

  7. analysis of HMGCLL1 as an extramitochondrial human 3-hydroxy-3-methylglutaryl-CoA lyase and comparison with MHGCL

  8. An alternative transcript of HMGCS2 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.

  9. 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.

  10. 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.

  11. 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).

  12. The peroxisomal enzyme forms a covalently linked dimeric species upon crosslinking with dibromopropanone or o-phenylenedimaleimide . Cysteine-323 is required for intersubunit covalent crosslinking.

  13. A role is suggested for arginine-41 in deprotonation or enolization of acetyldithio-CoA, implicating this residue in the HMG-CoA cleavage reaction chemistry that leads to acetyl-CoA product formation.

  14. Data describe the DNA mutational analysis of 3-hydroxy-3-methylglutaryl-coenzyme A lyase.

  15. Exon 2 of HL skipping led to the loss of beta-sheet 1, and the skipping of exons 2 and 3 caused the disappearance of alpha helix 1 and beta-sheets 1 and 2

  16. Crystal structure of human 3-hydroxy-3-methylglutaryl-CoA Lyase

  17. findings of common mutations in HMGCL have direct implications on rapid molecular diagnosis, prenatal and pre-implantation diagnosis and population based prevention programs directed towards 3HMG in Saudi Arabia.

  18. HMG-CoA located on the surface of the enzyme implicates Asn(311) and Lys(313) in substrate binding by establishing polar contacts with phosphate and ribose groups of adenosine, and Lys(48) by contacting the carboxyl group of the panthotenic acid moiety.

  19. The unique HMGCL gene mutations exist in Taiwanese 3-hydroxy-3-methylglutaryl CoA lyase deficiency deficiency patients.

  20. Finding that all identified missense mutations cause a >95% decrease in the enzyme activity, indicates that the disease appears only in very severe genotypes.

HMGCL profil antigène

Antigen Summary

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.

Gene names and symbols associated with HMGCL

  • 3-hydroxymethyl-3-methylglutaryl-CoA lyase S homeolog (hmgcl.S) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-CoA lyase (HMGCL) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (AFUA_7G01720) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (NFIA_114450) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (ACLA_065820) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (AOR_1_910074) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (PMAA_004600) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (TSTA_103060) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (ARB_04874) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (TRV_06573) anticorps
  • 3-hydroxy-3-methylglutaryl-Coenzyme A lyase (Hmgcl) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-CoA lyase (Hmgcl) anticorps
  • 3-hydroxymethyl-3-methylglutaryl-CoA lyase (hmgcl) anticorps
  • Afu7g01720 anticorps
  • AO090038000541 anticorps
  • AW476067 anticorps
  • HL anticorps
  • zgc:56248 anticorps

Protein level used designations for HMGCL

3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase , 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria) , 3-hydroxy-3-methylglutarate-CoA lyase , HL , HMG-CoA lyase , hydroxymethylglutaryl-CoA lyase, mitochondrial , 3-hydroxy-3-methylglutaryl-CoA lyase , hydroxymethylglutaricaciduria , mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase , 3-hydroxy-3-methylglutaryl-Coenzyme A lyase

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