PLOD1 Chromosome 1

Procollagen-lysine,2-oxoglutarate 5-dioxygenase 1
150 variants 150 Health Risk

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What This Gene Does
Lysyl hydroxylase is a membrane-bound homodimeric protein localized to the cisternae of the endoplasmic reticulum. The enzyme (cofactors iron and ascorbate) catalyzes the hydroxylation of lysyl residues in collagen-like peptides. The resultant hydroxylysyl groups are attachment sites for carbohydrates in collagen and thus are critical for the stability of intermolecular crosslinks. Some patients with Ehlers-Danlos syndrome type VI have deficiencies in lysyl hydroxylase activity. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2015]
Gene Info
Gene Group
Procollagen-lysine,2-oxoglutarate 5-dioxygenase family
Locus Type
gene with protein product
Location
1p36.22
Ensembl
ENSG00000083444
Associated Conditions (11)
Ehlers-Danlos syndrome
kyphoscoliotic type 1
Familial thoracic aortic aneurysm and aortic dissection
PLOD1-related disorder
Cardiovascular phenotype
Keratoconus
Thyroid cancer
nonmedullary
1
6 conditions
8 conditions
Key Variants
RS1032781250
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS112250644
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS11553676
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1176136454
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1264171984
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
Health Risk
RS1273287120
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1310692062
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
Health Risk
RS138490756
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1391173198
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, Familial thoracic aortic aneurysm and aortic dissection, kyphoscoliotic type 1
Health Risk
RS139869965
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS140159343
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS140513387
Conflicting classifications of pathogenicity
Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
All Variants (150)
RSID Category Clinical Significance Conditions
RS1032781250 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS112250644 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS11553676 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS1176136454 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS1264171984 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS1273287120 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS1310692062 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS138490756 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS1391173198 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, Familial thoracic aortic aneurysm and aortic dissection, kyphoscoliotic type 1
RS139869965 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS140159343 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS140513387 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS140758113 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS142710681 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS142934642 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS143114026 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS144226170 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS144439284 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS144465849 Health Risk Conflicting classifications of pathogenicity
RS145482271 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS146092290 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS147940796 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS147980436 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS149425237 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS1553136318 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS1569708580 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS199669594 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS199946373 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS200131516 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS200395169 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS201661871 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS201888323 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS369263247 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS370305686 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS370358836 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS371488160 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS372579008 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS373165011 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS373324035 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS373471550 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS375416784 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS376288573 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS377406897 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS534978828 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, PLOD1-related disorder
RS538255620 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS541901674 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS557317492 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS565216977 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Ehlers-Danlos syndrome
RS565513365 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
RS565765056 Health Risk Conflicting classifications of pathogenicity Ehlers-Danlos syndrome, kyphoscoliotic type 1, Familial thoracic aortic aneurysm and aortic dissection
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