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UniProt AC
UniProt Status
Protein Name
Organism
PMID
Article Title
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Annotation
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Q9WVJ9 ;undefined 29931197Decreased mitochondrial respiration in aneurysmal aortas of Fibulin-4 mutant mice is linked to PGC1A regulation.[Function]Protein/gene_name: FBLN-4.
Function: In mutant mice with 4-fold reduction in FBLN-4 protein, altered mitochondrial function and metabolic dysregulation.
Additional Annotation: Decreased mitochondrial respiration without changes in expression or activity of mitochondrial complex proteins and increased reactive oxygen species. Mediated via increased TGF-beta signaling and reduced PGC1-a levels.
Disease: Thoracic aortic aneurysm.
swapna menon0000-0002-0429-24722024-7-14
Q9WVJ9 ;undefined 30844428Fibulin-4 deficiency differentially affects cytoskeleton structure and dynamics as well as TGFβ signaling.[Function]Protein/gene_name: FBLN4.
Function: Influences cytoskeletal structure and TGF-beta signaling in vascular smooth muscle cells.
Additional Annotation:
Disease: Aortic aneurysm.
swapna menon0000-0002-0429-24722024-7-14
Q9WVJ9 ;undefined 31548410Fibulin-4 exerts a dual role in LTBP-4L-mediated matrix assembly and function.[Function][Interaction]Protein/gene_name: FBLN4.
Function: Critical to elastogenesis.
Additional Annotation: FBLN-4 multimers, not monomers, act as extracellular chaperones on LTBP-4L, inducing a stable conformational change in the latter. This conformational change promotes deposition of tropelastin on elongated LTBP-4L fibrils, as well as binding of LTPB-4L to other elastogenic proteins like fibronectin and fibrillin-1.
swapna menon0000-0002-0429-24722024-7-14
Q9WVJ9 ;undefined 33239290A matricellular protein fibulin-4 is essential for the activation of lysyl oxidase.[Function][Pathology & Biotech]Protein/gene_name: FBLN4.
Function: Has a role in extracellular matrix assembly, where it is essential for the activity of LOX (Lysyl oxidase enzyme). Required for the transfer of copper ion from copper transporter ATP7A to LOX in the trans-golgi network. This is an important step in the formation of lysine tyrosyl quinone (LTQ), an essential LOX cofactor.
swapna menon0000-0002-0429-24722024-7-14