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HMG CoA lyase deficiency

Known as: 3-Hydroxy-3-Methylglutaryl-Coenzyme A Lyase Deficiency, Hydroxymethylglutaryl-CoA Lyase Deficiency, 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY 
A rare genetic inherited disorder characterized by inability of the body to process the amino acid leucine. Signs and symptoms include vomiting… 
National Institutes of Health

Related topics

Related topics

5 relations

Broader (2)

Acetyl-CoA C-AcetyltransferaseAmino Acid Metabolism, Inborn Errors
HMGCL geneHereditary DiseasesHydroxymethylglutaryl CoA lyase deficiency:Imp:Pt:Bld.dot:Nom

Papers overview

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Highly Cited
2007
Highly Cited
2007
HMGB1 is a cofactor in mammalian base excision repair.
Highly Cited
2004
Highly Cited
2004
Silencing of hydroperoxide lyase and allene oxide synthase reveals substrate and defense signaling crosstalk in Nicotiana attenuata.
The fatty acid hydroperoxide (HP) substrates required for the biosynthesis of jasmonic acid (JA) and green leaf volatiles (GLVs… 
Highly Cited
2002
Highly Cited
2002
A spectrophotometric assay for allicin, alliin, and alliinase (alliin lyase) with a chromogenic thiol: reaction of 4-mercaptopyridine with thiosulfinates.
Highly Cited
2002
Highly Cited
2002
Acetylation regulates the DNA end-trimming activity of DNA polymerase beta.
Highly Cited
1999
Highly Cited
1999
A nifS-like gene, csdB, encodes an Escherichia coli counterpart of mammalian selenocysteine lyase. Gene cloning, purification, characterization and preliminary x-ray crystallographic studies.
Selenocysteine lyase is a pyridoxal 5'-phosphate (PLP)-dependent enzyme that catalyzes the exclusive decomposition of L… 
Highly Cited
1997
Highly Cited
1997
Pectate lyase PelI of Erwinia chrysanthemi 3937 belongs to a new family
Erwinia chrysanthemi 3937 secretes five major isoenzymes of pectate lyases encoded by the pel4, pelB, pelC, pelD, and pelE genes… 
Highly Cited
1992
Highly Cited
1992
Cloning and sequencing of Escherichia coli ubiC and purification of chorismate lyase
In Escherichia coli, chorismate lyase catalyzes the first step in ubiquinone biosynthesis, the conversion of chorismate to 4… 
Highly Cited
1989
Highly Cited
1989
Cloning and analysis of the Neurospora crassa gene for cytochrome c heme lyase.
Highly Cited
1987
Highly Cited
1987
Characterization of the Erwinia carotovora pelB gene and its product pectate lyase
The pelB gene encodes pectate lyase B, one of three pectate lyases identified in Erwinia carotovora EC. Pectate lyase B was… 
Highly Cited
1976
Highly Cited
1976
The urinary organic acid profile associated with 3-hydroxy-3-methylglutaric aciduria.
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