Link to this page
Biological Pathway Taxonomy
Last uploaded:
March 30, 2022
Jump to:
| Id | urn:agi-pathway:uuid-620aa17c-c871-4b40-aea4-0d19c69a3927
urn:agi-pathway:uuid-620aa17c-c871-4b40-aea4-0d19c69a3927
|
|---|---|
| Preferred Name | Skin Fibrosis |
| Synonyms |
PMID: 17133253
PathwayType: signaling
Organ: skin
Notes: Headnote: Systemic scleroderma, also known as systemic sclerosis (SSc) is a rare chronic autoimmune disease of undetermined cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints and visceral organs. Death usually occurs due to lung, heart, and kidney involvement. Skin fibrosis, the hallmark of SSc, is defined as excess deposition and accumulation of extracellular matrix, mainly type I collagen, in the dermis. Signaling description: Endothelial cells, T-helper (Th) cells, and B -cells produce several profibrotic factors including PDGF, VEGF, IGF1, AGT, EDN1, and TGFB. B-cells promote collagen production by fibroblasts through the release of cytokines or autoantibodies. Antifibrillin-1 antibodies, which are highly specific for systemic sclerosis (SSc), activate normal fibroblasts to differentiate into a profibrotic phenotype through the TGF-dependent pathway. Autoantibodies to PDGF receptors are stimulatory and induce the production of reactive oxygen species and the expression of collagen genes in fibroblasts. Extracellular matrix degradation is primarily regulated by matrix metalloproteinases (MMPs). Outcome effects: Auto anti-MMP1 Abs are suggested to inhibit MMP1 and are involved in the development of fibrosis in SSc; while, auto anti-MMP3 Abs are likely to inhibit MMP3 activity and thus promote fibrosis. Highlighted proteins: Proteins with increased expression or activity are highlighted in red, and proteins with decreased expression or activity are highlighted in blue. Mutated genes: Mutated genes are shown in white-out style.
Link: https://mammal-profservices.pathwaystudio.com/app/sd?urn=urn:agi-pathway:uuid-620aa17c-c871-4b40-aea4-0d19c69a3927
NodeType: Pathway
Tissue: epidermis
Pathway_Author: V. Sobolev ORCID:0000-0003-4779-156X
Organ_System: integumentary system
CellType: fibroblast
Source: Diseases
Description: Skin fibrosis, the hallmark of systemic sclerosis or scleroderma (SSc), is defined as excess deposition and accumulation of extracellular matrix, mainly type I collagen, in the dermis. Pathway is built manually using published studies.
See more
See less
|
| Type | http://www.w3.org/2002/07/owl#Class |
All Properties
| label | Skin Fibrosis
|
|---|---|
| prefLabel | Skin Fibrosis
|
| database_cross_reference |
PS:PathwayType
PS:Description
PS:Tissue
PS:Pathway_Author
PS:Link
PS:CellType
PS:Organ_System
PS:PMID
PS:NodeType
PS:Notes
PS:Organ
PS:Source
See more
See less
|
| notation | uuid-620aa17c-c871-4b40-aea4-0d19c69a3927
|
| id | urn:agi-pathway:uuid-620aa17c-c871-4b40-aea4-0d19c69a3927
|
| subClassOf |
urn:agi-folder:s
urn:agi-folder:systemic_scleroderma
urn:agi-folder:epidermis
urn:agi-folder:integumentary_system
|
| type | |
| has_exact_synonym | PMID: 17133253
PathwayType: signaling
Organ: skin
Notes: Headnote: Systemic scleroderma, also known as systemic sclerosis (SSc) is a rare chronic autoimmune disease of undetermined cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints and visceral organs. Death usually occurs due to lung, heart, and kidney involvement. Skin fibrosis, the hallmark of SSc, is defined as excess deposition and accumulation of extracellular matrix, mainly type I collagen, in the dermis. Signaling description: Endothelial cells, T-helper (Th) cells, and B -cells produce several profibrotic factors including PDGF, VEGF, IGF1, AGT, EDN1, and TGFB. B-cells promote collagen production by fibroblasts through the release of cytokines or autoantibodies. Antifibrillin-1 antibodies, which are highly specific for systemic sclerosis (SSc), activate normal fibroblasts to differentiate into a profibrotic phenotype through the TGF-dependent pathway. Autoantibodies to PDGF receptors are stimulatory and induce the production of reactive oxygen species and the expression of collagen genes in fibroblasts. Extracellular matrix degradation is primarily regulated by matrix metalloproteinases (MMPs). Outcome effects: Auto anti-MMP1 Abs are suggested to inhibit MMP1 and are involved in the development of fibrosis in SSc; while, auto anti-MMP3 Abs are likely to inhibit MMP3 activity and thus promote fibrosis. Highlighted proteins: Proteins with increased expression or activity are highlighted in red, and proteins with decreased expression or activity are highlighted in blue. Mutated genes: Mutated genes are shown in white-out style.
Link: https://mammal-profservices.pathwaystudio.com/app/sd?urn=urn:agi-pathway:uuid-620aa17c-c871-4b40-aea4-0d19c69a3927
NodeType: Pathway
Tissue: epidermis
Pathway_Author: V. Sobolev ORCID:0000-0003-4779-156X
Organ_System: integumentary system
CellType: fibroblast
Source: Diseases
Description: Skin fibrosis, the hallmark of systemic sclerosis or scleroderma (SSc), is defined as excess deposition and accumulation of extracellular matrix, mainly type I collagen, in the dermis. Pathway is built manually using published studies.
See more
See less
|
| treeView |
urn:agi-folder:s
urn:agi-folder:systemic_scleroderma
urn:agi-folder:epidermis
urn:agi-folder:integumentary_system
|
Add comment
| Delete | Subject | Author | Type | Created |
|---|---|---|---|---|
| No notes to display |