Lysyl hydroxylase: Information from Answers.com

procollagen-lysine 1, 2-oxoglutarate 5-dioxygenase 1
Identifiers
Symbol	PLOD1
Alt. symbols	LLH, PLOD
Entrez	5351
HUGO	9081
OMIM	153454
RefSeq	NM_000302
UniProt	Q02809
Other data
EC number	1.14.11.4
Locus	Chr. 1 p36.3-36.2

procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2
Identifiers
Symbol	PLOD2
Entrez	5352
HUGO	9082
OMIM	601865
RefSeq	NM_000935
UniProt	O00469
Other data
Locus	Chr. 3 q24

Lysyl hydroxylase (or procollagen-lysine 5-dioxygenase) is an oxygenase enzyme which catalyzes the hydroxylation of lysine to hydroxylysine. This reaction is necessary to the formation and stabilization of collagen. It takes place following protein synthesis (as a post-translational modification). The protein is a membrane-bound homodimeric enzyme that is localized to the cisternae (lumen) of the rough endoplasmic reticulum.

Pathology

A deficiency is associated with Ehlers-Danlos syndrome (type 6).

v • d • e Oxidoreductases: dioxygenases, including steroid hydroxylases (EC 1.14)

1.14.11: 2-oxoglutarate	Prolyl hydroxylase - Lysyl hydroxylase

1.14.13: NADH or NADPH	Flavin-containing monooxygenase (FMO1, FMO2, FMO3, FMO4, FMO5) - Nitric oxide synthase (NOS1, NOS2A, NOS3) - Cholesterol 7 alpha-hydroxylase - Methane monooxygenase - 3A4 - Lanosterol 14 alpha-demethylase

1.14.14: reduced flavin or flavoprotein	19A1 - 2D6 - 2E1

1.14.15: reduced iron-sulfur protein	11B1 - 11B2 - 11A1

1.14.16: reduced pteridine (BH4 dependent)	Phenylalanine hydroxylase - Tyrosine hydroxylase - Tryptophan hydroxylase

1.14.17: reduced ascorbate	Dopamine beta hydroxylase

1.14.18-19: other	Tyrosinase - Stearoyl-CoA desaturase-1

1.14.99 - miscellaneous	Cyclooxygenase - Heme oxygenase (HMOX1) - Squalene monooxygenase - 17A1 - 21A2

glycine→serine→	Serine hydroxymethyltransferase · Serine dehydratase glycine→creatine: Guanidinoacetate N-methyltransferase · Creatine kinase

alanine→	Alanine transaminase

cysteine→	D-cysteine desulfhydrase

threonine→	L-threonine dehydrogenase

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


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


asparagine→aspartate→	Asparaginase/Asparagine synthetase · Aspartate transaminase

see also disorders, intermediates

1-12	type I (COL1A1, COL1A2) · type II (COL2A1) · type III · type IV (COL4A1, COL4A2, COL4A3, COL4A4, COL4A5, COL4A6) · type V (COL5A1, COL5A2, COL5A3) · type VI (COL6A1, COL6A2, COL6A3) · type VII (COL7A1) · type VIII (COL8A1, COL8A2) · type IX (COL9A1, COL9A2, COL9A3) · type X (COL10A1) · type XI (COL11A1, COL11A2) · type XII (COL12A1)

13-29	COL13A1 · COL14A1 · COL15A1 · COL16A1 · COL17A1 · type XVIII (COL18A1, Endostatin) · COL19A1 · COL20A1 · COL21A1 · COL22A1 · COL23A1 · COL24A1 · COL25A1 · COL26A1 · COL27A1 · COL28A1 · COL29A1

Enzymes	Prolyl hydroxylase/Lysyl hydroxylase · Cartilage associated protein/Leprecan · ADAMTS2 · Procollagen peptidase · Lysyl oxidase

Other

Epithelial keratins (soft alpha-keratins)	type I/chromosome 17 (10, 12, 13, 14, 15, 16, 17, 19, 20) · chromosome 12 (18) · none (21) type II/chromosome 12 (1, 2A, 3, 4, 5, 6A, 6B, 7, 8, 9)

Hair keratins (hard alpha-keratins)	type I/chromosome 17 (31, 32, 33A, 33B, 34, 35, 36, 37, 38) type II/chromosome 12 (81, 82, 83, 84, 85, 86)

Ungrouped alpha	chromosome 17 (23, 24, 25, 26, 27, 28, 39, 40) chromosome 12 (71, 72, 73, 74, 75, 76, 77, 78, 79, 80)

Not alpha	Beta-keratin

Other

see also diseases

This oxidoreductase article is a stub. You can help Wikipedia by expanding it.

This entry is from Wikipedia, the leading user-contributed encyclopedia. It may not have been reviewed by professional editors (see full disclaimer)

	What is aldosterone state in 11 hydroxylase dediciency?
	Does Tryptophan hydroxylase regulates the amount of enzyme in the serotonin biosynthesis?
	What is a reaction for ascorbic acid and the Iron 3 cofactor of the hydroxylase?