Procollagen N-Endopeptidase (Procollagen Peptidase)

An extracellular endopeptidase which excises a block of peptides at the amino terminal, nonhelical region of the procollagen molecule with the formation of collagen. Absence or deficiency of the enzyme causes accumulation of procollagen which results in the inherited connective tissue disorder--dermatosparaxis. EC 3.4.24.14.

Also Known As:

Networked: 13 relevant articles (0 outcomes, 0 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Becker, Marte A: 1 article (01/2018)
2.	Bemelman, Willem A: 1 article (01/2018)
3.	Buskens, Christianne J: 1 article (01/2018)
4.	D'Haens, Geert R: 1 article (01/2018)
5.	Löwenberg, Mark: 1 article (01/2018)
6.	Meijer, Sybren L: 1 article (01/2018)
7.	Ponsioen, Cyriel Y: 1 article (01/2018)
8.	Steenkamer, Jessica: 1 article (01/2018)
9.	Wildenberg, Manon E: 1 article (01/2018)
10.	de Bruyn, Jessica R: 1 article (01/2018)

Related Diseases

1.	Autosomal Recessive Type VII Ehlers-Danlos Syndrome 04/01/1993 - "This disease, resulting from a defect in procollagen peptidase, resembles dermatosparaxis in animals. " 01/01/2001 - "ADAMTS2 is a procollagen N-proteinase, and the mutations of its gene are responsible for Human Ehlers-Danlos syndrome type VII C and bovine dermatosparaxis. " 02/01/1980 - "Dermatosparaxis is a connective tissue disease, primarily of sheep and cattle, that results from deficient activity of the NH2-terminal procollagen peptidase. " 04/24/1998 - "Dermatosparaxis is a recessive disorder of animals (including man) which is caused by mutations in the gene for the enzyme procollagen N-proteinase and is characterised by extreme skin fragility. " 02/01/1977 - "In lathyrism, the toxic agent directly blocks the development of the transverse links in the collagen fibre, while in bovine dermatosparaxis there is a deficiency of procollagen peptidase. "
2.	Ehlers-Danlos Syndrome (Syndrome, Ehlers-Danlos) 01/01/1973 - "Procollagen peptidase deficiency in a form of the Ehlers-Danlos syndrome." 01/01/2001 - "ADAMTS2 is a procollagen N-proteinase, and the mutations of its gene are responsible for Human Ehlers-Danlos syndrome type VII C and bovine dermatosparaxis. " 04/15/1990 - "The cause of the Ehlers-Danlos syndrome Type VII (EDS VII) is considered to be defective removal of the amino-terminal propeptide (N-propeptide) of Type I procollagen due to deficiency of procollagen N-proteinase, the enzyme responsible for the normal proteolytic excision of this precursor-specific domain. " 02/01/1977 - "Of the many clinical forms of the Ehlers-Danlos syndrome, some are due to a deficiency of lysine oxidase, others to deficiency of lysine hydroxylase and still others to procollagen peptidase deficiency. "
3.	Type IV Ehlers-Danlos Syndrome 01/01/1995 - "Recent advances in the molecular analysis of EDS have identify defects responsible for EDS VI (homozygous and compound heterozygous mutations in the lysyl-hydroxylase gene), EDS VIIA and EDS VIIB (mutations in the type I collagene genes), EDS VIIC (deficiency of procollagen N-proteinase), EDS IX (mutations in the MNK gene), and EDS IV (mutations in the type III collagen gene). " 09/01/1993 - "Recent advances in the molecular analysis of EDS have identified defects responsible for EDS IV (mutations in the type III collagen gene), EDS VI (homozygous and compound heterozygous mutations in the lysyl hydroxylase gene), EDS VIIA and VIIB (mutations in the type I collagen genes), EDS VIIC (deficiency of procollagen N-proteinase), and EDS IX (decreased lysyl oxidase activity). "
4.	Autosomal Dominant Type VII Ehlers-Danlos Syndrome 01/01/1995 - "Recent advances in the molecular analysis of EDS have identify defects responsible for EDS VI (homozygous and compound heterozygous mutations in the lysyl-hydroxylase gene), EDS VIIA and EDS VIIB (mutations in the type I collagene genes), EDS VIIC (deficiency of procollagen N-proteinase), EDS IX (mutations in the MNK gene), and EDS IV (mutations in the type III collagen gene). " 09/01/1993 - "Recent advances in the molecular analysis of EDS have identified defects responsible for EDS IV (mutations in the type III collagen gene), EDS VI (homozygous and compound heterozygous mutations in the lysyl hydroxylase gene), EDS VIIA and VIIB (mutations in the type I collagen genes), EDS VIIC (deficiency of procollagen N-proteinase), and EDS IX (decreased lysyl oxidase activity). "
5.	Occipital horn syndrome 01/01/1995 - "Recent advances in the molecular analysis of EDS have identify defects responsible for EDS VI (homozygous and compound heterozygous mutations in the lysyl-hydroxylase gene), EDS VIIA and EDS VIIB (mutations in the type I collagene genes), EDS VIIC (deficiency of procollagen N-proteinase), EDS IX (mutations in the MNK gene), and EDS IV (mutations in the type III collagen gene). " 09/01/1993 - "Recent advances in the molecular analysis of EDS have identified defects responsible for EDS IV (mutations in the type III collagen gene), EDS VI (homozygous and compound heterozygous mutations in the lysyl hydroxylase gene), EDS VIIA and VIIB (mutations in the type I collagen genes), EDS VIIC (deficiency of procollagen N-proteinase), and EDS IX (decreased lysyl oxidase activity). "

Related Drugs and Biologics

1.	2-Oxoglutarate 5-Dioxygenase Procollagen-Lysine (Lysyl Hydroxylase)
2.	Enzymes
3.	Collagen Type III (Type III Collagen)
4.	Procollagen Type I
5.	Collagen Type I (Type I Collagen)
6.	Serine (L-Serine)
7.	Messenger RNA (mRNA)
8.	Procollagen
9.	Oxidoreductases (Dehydrogenase)
10.	Protein-Lysine 6-Oxidase (Lysyl Oxidase)