M. Bishr Omary

CPS1: a multipurpose mitochondrial enzyme, bile protein, acute liver injury biomarker, and cytokine

Lu Chen, Pei Li, Min-Jung Park, Z. Chen et autres

Carbamoyl phosphate synthetase 1 (CPS1) is primarily expressed in hepatocytes as a highly abundant mitochondrial matrix enzyme that catalyses the first step of the urea cycle that leads to renal nitrogen disposal. CPS1 is a member of the CPS family that manifests …

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Preclinical validation of PKC412 as a therapy candidate for epidermolysis bullosa simplex across multiple keratin pathogenic variants

Henriette Jopp, A Kraft, Bernd Hoffmann, Rudolf Merkel et autres

BACKGROUND: Epidermolysis bullosa simplex (EBS) is a hereditary skin fragility disorder caused by missense pathogenic variants in KRT5 or KRT14. These variants trigger the collapse of the cytoskeleton into cytoplasmic protein aggregates, rendering the epidermis highly susceptible to mechanical stress, leading to …

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The Histamine Pathway is a Target to Treat Hepatic Experimental Erythropoietic Protoporphyria

Ning Kuo, Pei Li, Juliana Bragazzi Cunha, Lu Chen et autres

Background & Aims Erythropoietic protoporphyria (EPP) is caused by mutations in ferrochelatase, which inserts iron into protoporphyrin-IX (PP-IX) to generate heme. EPP is characterized by PP-IX accumulation, skin photosensitivity, cholestasis, and end-stage liver disease. Despite available drugs that address photosensitivity, treatment of …

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World Gastroenterology Organisation - Gut commentary series on digestive health and climate change

M. Bishr Omary, Desmond Leddin, Geoffrey Metz, Andrew Veitch et autres

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Gastroenterology climate action opportunities via education, empowerment of trainees and research

Aasma Shaukat, Brijen J. Shah, Cassandra Fritz, M. Bishr Omary

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Posttranslational modifications of keratins and their associated proteins as therapeutic targets in keratin diseases

Pei Li, Katrin Rietscher, Thomas M. Magin, M. Bishr Omary

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Regulation of epithelial transitional states in murine and human pulmonary fibrosis

Fa Wang, Christopher Ting, Kent Riemondy, Michael Douglas et autres

Idiopathic pulmonary fibrosis (IPF) is a progressive scarring disease arising from impaired regeneration of the alveolar epithelium after injury. During regeneration, type 2 alveolar epithelial cells (AEC2s) assume a transitional state that upregulates multiple keratins and ultimately differentiate into AEC1s. In IPF, …

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Painting and dissecting Epidermolysis Bullosa Simplex-associated keratin aggregates

Katrin Rietscher, Melanie Homberg, Ilya Kovalenko, Jonathan Z. Sexton et autres

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Hypoosmosis alters hepatocyte mitochondrial morphology and induces selective release of carbamoyl phosphate synthetase 1

Pei Li, Ning Kuo, R. D. Patel, M. Bishr Omary

Exposure of isolated mouse livers, primary cultured hepatocytes, or mice to hypoosmosis/hyponatremia conditions induces significant mitochondrial shape alterations accompanied by preferential release of the mitochondrial matrix protein CPS1, a urea cycle enzyme. In contrast, the intermembrane space protein, cytochrome c, and two …

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Deacetylation via SIRT2 prevents keratin-mutation-associated injury and keratin aggregation

Jingyuan Sun, Pei Li, Honglian Gui, Laure Rittié et autres

Keratin (K) and other intermediate filament (IF) protein mutations at conserved arginines disrupt keratin filaments into aggregates and cause human epidermolysis bullosa simplex (EBS; K14-R125C) or predispose to mouse liver injury (K18-R90C). The challenge for more than 70 IF-associated diseases is the …

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Hepatocyte-specific loss of LAP2α protects against diet-induced hepatic steatosis, steatohepatitis, and fibrosis in male mice

Kapil K. Upadhyay, Eun-Young Choi, Roland Foisner, M. Bishr Omary et autres

The nuclear envelope and lamina regulate lipid metabolism and susceptibility to nonalcoholic steatohepatitis (NASH), but the role of the nuclear lamin-binding protein LAP2α in NASH has not been explored. Our data demonstrate that hepatocyte-specific loss of LAP2α protects against diet-induced hepatic steatosis, …

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Presentation of the AGA William Beaumont Prize in Gastroenterology to Anil K. Rustgi, MD

Timothy C. Wang, M. Bishr Omary

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