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Molecular Defect

Mitochondrial Antibodies TGF Dysregulations

Mitochondrial Antibodies

Mitochondrial Antibodies occur in about 95% of people who have primary biliary cirrhosis (PBC), but only 3% of people who have PBC have systemic scleroderma. (Also see What is Scleroderma? and Types of Scleroderma)

United Mitochondrial Disease Foundation. Mitochondrial diseases result from failures of the mitochondria, specialized compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. United Mitochondrial Disease Foundation.

Pyruvate kinase M2 and the mitochondrial ATPase Inhibitory Factor 1 provide novel biomarkers of dermatomyositis: a metabolic link to oncogenesis. Reverse phase protein microarrays identified the glycolysis promoting PKM2 and IF1 proteins as specific biomarkers of dermatomyositis, providing a biochemical link of this inflammatory myopathies with oncogenesis. PubMed, J Transl Med, 2017 Feb 10;15(1):29.

Low Cytochrome Oxidase 1 Links Mitochondrial Dysfunction to Atherosclerosis in Mice and Pigs. Low mitochondria–encoded cytochrome oxidase 1 is related to mitochondrial dysfunction, oxidative stress and atherosclerosis and plaque complexity. PubMed, PLoS One. 2017 Jan 25;12(1):e0170307.

The Fingerprint of Antimitochondrial Antibodies and the Etiology of Primary Biliary Cholangitis (PBC). A molecular understanding of the conformation of xenobiotic modified PDC-E2 is critical for understanding xenobiotic modification and loss of tolerance in PBC with widespread implications for a role of environmental chemicals in the induction of autoimmunity. PubMed, Hepatology, 01/18/2017. (Also see Liver Involvement)

TGF Dysregulations

Some researchers suspect that a molecular defect in TGFbeta/SMAD may play a role in the development of scleroderma fibrosis.

Pan peroxisome proliferator-activated receptors (PPAR) agonist IVA337 is effective in prevention and treatment of experimental skin fibrosis. These findings indicate that simultaneous activation of all three PPAR isoforms exerts a dampening effect on inflammation and fibrosis, making IVA337 a potentially effective therapeutic candidate in the treatment of fibrotic diseases including systemic sclerosis. PubMed, Ann Rheum Dis, 2016 Dec;75(12):2175-2183. (Also see Skin Fibrosis)

Tribbles homologue 3 stimulates canonical TGF-ß signalling to regulate fibroblast activation and tissue fibrosis. The present study characterises TRB3 as a novel profibrotic mediator in systemic sclerosis. PubMed, Ann Rheum Dis, 2016 Mar;75(3):609-16. (Also see Fibroblasts)

Transforming growth factor-ß increases interleukin-13 synthesis via GATA-3 transcription factor in T-lymphocytes from patients with systemic sclerosis (SSc). These results demonstrate that TGF-ß upregulates IL-13 synthesis through GATA-3 expression in the T lymphocytes of patients with SSc, confirming that the GATA-3 transcription factor can be regarded as a novel therapeutic target in patients with SSc. PubMed, Arthritis Res Ther, 2015 Jul 31;17:196. (Also see Interleukins)

Study Shows Treatment with Fresolimumab Able to Reduce Skin Scarring in Systemic Scleroderma patients. Fresolimumab, sponsored by Genzyme, a drug agent that targets a chemical mediator in the body called TGF-beta, is able to block scarring which could mean a major treatment advance for scarring-mediated organ dysfunction. Scleroderma News, 06/23/2015. (Also see Skin Fibrosis and Scleroderma Clinical Trials)

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