3-hydroxy-3-methylglutaryl-CoA lyase

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

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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 A/Aldolase B - 2-hydroxyphytanoyl-CoA lyase - Threonine aldolase

4.1.3: Oxo-acid-lyases	3-hydroxy-3-methylglutaryl-CoA lyase

v • d • e Metabolism: lipid metabolism - ketones/mevalonate pathway/cholesterol synthesis enzymes

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

To Cholesterols	Dimethylallyltranstransferase - Geranyl pyrophosphate - Farnesyl-diphosphate farnesyltransferase - Squalene monooxygenase - Lanosterol synthase - CYP51A1

To Bile acids	Cholesterol 7 alpha-hydroxylase

see also intermediates

v • d • e

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)

glycine→serine→	Serine hydroxymethyltransferase - Serine dehydratase

alanine→	Alanine transaminase

cysteine→	D-cysteine desulfhydrase

threonine→	L-threonine dehydrogenase


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


PHENYLALANINE→tyrosine→	Phenylalanine hydroxylase - Tyrosine aminotransferase - 4-Hydroxyphenylpyruvate dioxygenase - Homogentisate 1,2-dioxygenase - Fumarylacetoacetate hydrolase