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

The protein encoded by HMGCL belongs to the HMG-CoA lyase family. Additionally we are shipping HMGCL Antibodies (110) and HMGCL Proteins (16) and many more products for this protein.

list all ELISA KIts Gene Name GeneID UniProt
HMGCL 15356 P38060
HMGCL 3155 P35914
HMGCL 79238 P97519
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Top HMGCL ELISA Kits at antibodies-online.com

Showing 4 out of 7 products:

Catalog No. Reactivity Sensitivity Range Images Quantity Supplier Delivery Price Details
Cow 5.8 pg/mL 23.5-1500 pg/mL Typical standard curve 96 Tests Log in to see 15 to 18 Days
$1,026.67
Details
Human < 0.188 ng/mL 0.313 ng/mL - 20 ng/mL   96 Tests Log in to see 11 to 18 Days
$810.17
Details
Rat < 0.094 ng/mL 0.156 ng/mL - 10 ng/mL   96 Tests Log in to see 11 to 18 Days
$810.17
Details
Mouse < 46.9 pg/mL 78 pg/mL - 5000 pg/mL   96 Tests Log in to see 11 to 18 Days
$810.17
Details

More ELISA Kits for HMGCL Interaction Partners

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

  1. 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)

  2. 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.

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

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

  5. 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

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

  7. 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.

  8. 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.

  9. 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.

  10. 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).

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

  12. 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.

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

  14. 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

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

  16. 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.

  17. 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.

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

  19. 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 Antigen Profile

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

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

GENE ID SPECIES
432233 Xenopus laevis
456624 Pan troglodytes
710833 Macaca mulatta
3504221 Aspergillus fumigatus Af293
4589480 Neosartorya fischeri NRRL 181
4704707 Aspergillus clavatus NRRL 1
5997540 Aspergillus oryzae RIB40
7030025 Talaromyces marneffei ATCC 18224
8102925 Talaromyces stipitatus ATCC 10500
9522067 Arthroderma benhamiae CBS 112371
9580122 Trichophyton verrucosum HKI 0517
100174514 Pongo abelii
15356 Mus musculus
3155 Homo sapiens
317658 Bos taurus
79238 Rattus norvegicus
396316 Gallus gallus
394190 Danio rerio
478187 Canis lupus familiaris
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