3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria)
Identifiers
Symbol	HMGCL
Entrez	3155
HUGO	5005
OMIM	246450
RefSeq	NM_000191
UniProt	P35914
Other data
EC number	4.1.3.4
Locus	Chr. 1 p36.1-p35

3-hydroxy-3-methylglutaryl-CoA lyase (or HMG-CoA lyase) plays an essential role in breaking down dietary proteins and fats for energy.

Specifically, the enzyme is responsible for processing the amino acid leucine.

[edit] Pathology

Mutations in the HMGCL gene cause 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.

[edit] External links

• MeSH 3-hydroxy-3-methylglutaryl-coenzyme+A+lyase

This lyase article is a stub. You can help Wikipedia by expanding it. v • d • e

v • d • e Carbon-carbon lyases (EC 4.1) 4.1.1: Carboxy-lyases Pyruvate decarboxylase · Oxaloacetate decarboxylase · Acetoacetate decarboxylase · Malonyl-CoA decarboxylase · Glutamate decarboxylase · Ornithine decarboxylase · Lysine decarboxylase · Phosphoribosylaminoimidazole carboxylase · Histidine decarboxylase · Uridine monophosphate synthetase/Orotidine 5'-phosphate decarboxylase · Aromatic L-amino acid decarboxylase · Phosphoenolpyruvate carboxylase · Pyrophosphomevalonate decarboxylase · Uroporphyrinogen III decarboxylase · RuBisCO · Phosphoenolpyruvate carboxykinase · Adenosylmethionine decarboxylase 4.1.2: Aldehyde-lyases Aldolase (Aldolase A, Aldolase B, Aldolase C) · 2-hydroxyphytanoyl-CoA lyase · Threonine aldolase 4.1.3: Oxo-acid-lyases 3-hydroxy-3-methylglutaryl-CoA lyase 4.1.99: Other Tryptophanase

v • d • e Metabolism: lipid metabolism · ketones/cholesterol synthesis enzymes/steroid metabolism Mevalonate pathway To HMG-CoA Acetyl-Coenzyme A acetyltransferase · HMG-CoA synthase (regulated step) Ketogenesis HMG-CoA lyase · 3-hydroxybutyrate dehydrogenase · Thiophorase To Mevalonic acid HMG-CoA reductase To DMAPP Mevalonate kinase · Phosphomevalonate kinase · Pyrophosphomevalonate decarboxylase · Isopentenyl-diphosphate delta isomerase Geranyl- Dimethylallyltranstransferase · Geranyl pyrophosphate To cholesterol To lanosterol Farnesyl-diphosphate farnesyltransferase · Squalene monooxygenase · Lanosterol synthase 7-Dehydrocholesterol path Lanosterol 14α-demethylase · Sterol-C5-desaturase-like · 7-Dehydrocholesterol reductase Desmosterol path 24-dehydrocholesterol reductase To Bile acids Cholesterol 7α-hydroxylase · Sterol 27-hydroxylase Steroidogenesis To pregnenolone Side-chain cleavage To corticosteroids aldosterone: 18-hydroxylase cortisol/cortisone: 17α-hydroxylase · 11β dehydrogenase (HSD11B1, HSD11B2) both: 3β dehydrogenase · 21α-hydroxylase · 11β-hydroxylase To sex hormones To androgens 17α-hydroxylase/17,20 lyase · 3β dehydrogenase · 17β dehydrogenase · 5α reductase (1,2) To estrogens Aromatase Other/ungrouped Steroid sulfatase · Steroidogenic acute regulatory protein see also intermediates, disorders

v • d • e Metabolism: amino acid metabolism · synthesis and catabolism enzymes (essential in CAPS) K→acetyl-CoA LYSINE→ Saccharopine dehydrogenase · Glutaryl-CoA dehydrogenase LEUCINE→ Branched chain aminotransferase · Branched-chain alpha-keto acid dehydrogenase complex · Isovaleryl coenzyme A dehydrogenase · Methylcrotonyl-CoA carboxylase · Methylglutaconyl-CoA hydratase · 3-hydroxy-3-methylglutaryl-CoA lyase TRYPTOPHAN→ Indoleamine 2,3-dioxygenase/Tryptophan 2,3-dioxygenase · Arylformamidase · Kynureninase · 3-hydroxyanthranilate oxidase · Aminocarboxymuconate-semialdehyde decarboxylase · Aminomuconate-semialdehyde dehydrogenase PHENYLALANINE→tyrosine→ (see below) G G→pyruvate →citrate glycine→serine→ Serine hydroxymethyltransferase · Serine dehydratase glycine→creatine: Guanidinoacetate N-methyltransferase · Creatine kinase alanine→ Alanine transaminase cysteine→ D-cysteine desulfhydrase threonine→ L-threonine dehydrogenase G→glutamate→ α-ketoglutarate HISTIDINE→ Histidine ammonia-lyase · Urocanate hydratase · Formiminotransferase cyclodeaminase proline→ Proline oxidase · Pyrroline-5-carboxylate reductase · 1-Pyrroline-5-carboxylate dehydrogenase/ALDH4A1 · PYCR1 arginine→ Ornithine aminotransferase · Ornithine decarboxylase · Agmatinase →alpha-ketoglutarate→TCA Glutamate dehydrogenase Other cysteine+glutamate→glutathione: Gamma-glutamylcysteine synthetase · Glutathione synthetase · Gamma-glutamyl transpeptidase glutamate→glutamine: Glutamine synthetase · Glutaminase G→propionyl-CoA→ succinyl-CoA VALINE→ Branched chain aminotransferase · Branched-chain alpha-keto acid dehydrogenase complex · Enoyl-CoA hydratase · 3-hydroxyisobutyryl-CoA hydrolase · 3-hydroxyisobutyrate dehydrogenase · Methylmalonate semialdehyde dehydrogenase ISOLEUCINE→ Branched chain aminotransferase · Branched-chain alpha-keto acid dehydrogenase complex · 3-hydroxy-2-methylbutyryl-CoA dehydrogenase METHIONINE→ generation of homocysteine: Methionine adenosyltransferase · Adenosylhomocysteinase regeneration of methionine: MTR/Homocysteine methyltransferase · Betaine-homocysteine methyltransferase conversion to cysteine: Cystathionine beta synthase · Cystathionine gamma-lyase THREONINE→ Threonine aldolase →succinyl-CoA→TCA Propionyl-CoA carboxylase · Methylmalonyl CoA epimerase · Methylmalonyl-CoA mutase G→fumarate PHENYLALANINE→tyrosine→ Phenylalanine hydroxylase · Tyrosine aminotransferase · 4-Hydroxyphenylpyruvate dioxygenase · Homogentisate 1,2-dioxygenase · Fumarylacetoacetate hydrolase tyrosine→melanin: Tyrosinase G→oxaloacetate asparagine→aspartate→ Asparaginase/Asparagine synthetase · Aspartate transaminase see also disorders, intermediates