Results 61 to 70 of about 663 (143)

Mitochondrial membrane remodeling in stress adaptation: Lipid control of organelle quality

open access: yesProtein Science, Volume 35, Issue 8, August 2026.
Abstract Mitochondria respond to proteotoxic stress through the mitochondrial unfolded protein response, traditionally viewed as a transcriptional program that restores proteostasis by inducing chaperones and proteases. Emerging evidence indicates that mitochondrial membrane remodeling constitutes an additional adaptive component of this response ...
Lena J. Reichert   +2 more
wiley   +1 more source

Tafazzin regulates the function of lipopolysaccharide activated B lymphocytes in mice [PDF]

open access: yes, 2021
Abstract B lymphocytes are responsible for humoral immunity and play a key role in the immune response. Optimal mitochondrial function is required to support B cell activity during activation. We examined how deficiency of tafazzin, a cardiolipin remodeling enzyme required for mitochondrial function, alters the metabolic activity of B ...
Hana M. Zegallai   +7 more
openaire   +1 more source

Monolysocardiolipin in cultured fibroblasts is a sensitive and specific marker for Barth Syndrome

open access: yesJournal of Lipid Research, 2006
Barth Syndrome (BTHS) is an X-linked recessive disorder that results in abnormal metabolism of the mitochondrial phospholipid cardiolipin (CL). CLs are decreased and monolysocardiolipins (MLCLs), intermediates in CL metabolism, are increased in a variety
Michiel Adriaan van Werkhoven   +3 more
doaj   +1 more source

YAP‐activated lymph node fibroblasts contribute to extracellular matrix remodeling associated with extranodal extension of oral squamous cell carcinoma

open access: yesThe Journal of Pathology: Clinical Research, Volume 12, Issue 4, July 2026.
Abstract Desmoplasia, characterized by excessive expansion of cancer‐associated fibroblasts (CAFs) and aberrant extracellular matrix (ECM) deposition, is widely present especially in invasive cancers. The extranodal extension (ENE) of nodal metastasis involves the extension of invasive tumor cells through the lymph node capsule into the perinodal ...
Qian Zhang   +6 more
wiley   +1 more source

Role of Tafazzin and its isoforms on cardiolipin composition, cellular proliferation and gene expression [PDF]

open access: yes, 2023
Tafazzin is an acyltransferase with key functions in remodeling of the mitochondrial phospholipid cardiolipin (CL) by exchanging single fatty acids species in CL.
Jagirdar, Gayatri
core   +1 more source

Genotype–Phenotype Discordance in Cardiomyopathies: Pathophysiology, Clinical Expression, and Therapeutic Considerations

open access: yesHealth Science Reports, Volume 9, Issue 5, May 2026.
ABSTRACT Background Cardiomyopathies encompass a spectrum of myocardial disorders often attributed to underlying genetic mutations. However, genotype–phenotype discordance where the genetic profile does not align with the expected clinical presentation poses significant diagnostic, prognostic, and therapeutic challenges.
Abubakar Nazir   +9 more
wiley   +1 more source

New C-Terminal Conserved Regions of Tafazzin, a Catalyst of Cardiolipin Remodeling [PDF]

open access: yesOxidative Medicine and Cellular Longevity, 2019
Cardiolipin interacts with many proteins of the mitochondrial inner membrane and, together with cytochrome C and creatine kinase, activates them. It can be considered as an integrating factor for components of the mitochondrial respiratory chain, which provides for an efficient transfer of electrons and protons.
Gregory A. Shilovsky   +6 more
openaire   +2 more sources

Survival and Clinical Progression in Barth Syndrome: Insights From the Barth Syndrome Foundation's Database of 502 Affected Individuals

open access: yesJournal of Inherited Metabolic Disease, Volume 49, Issue 3, May 2026.
ABSTRACT Barth syndrome (BTHS; OMIM 302060) is an ultra‐rare, life‐limiting genetic disorder characterized by cardiomyopathy, skeletal muscle myopathy, neutropenia, gastrointestinal issues, and fatigue. Formal analyses of survival and clinical progression remain limited.
Kexin Fu   +7 more
wiley   +1 more source

Mitochondrial Homeostasis in Pancreatic β Cell Function: Mechanisms and Therapeutic Targets for Diabetes

open access: yesJournal of Diabetes, Volume 18, Issue 5, May 2026.
This review highlights mitochondrial dysfunction as a central driver of pancreatic β cell failure in diabetes, caused by disrupted mitochondrial quality control (MQC), oxidative stress, and impaired organelle communication. Emerging therapies, such as DRAK2 inhibitors and metabolic reprogramming agents, show promise in restoring β cell function by ...
Ruihan Li   +5 more
wiley   +1 more source

Role of Tafazzin in Hematopoiesis and Leukemogenesis [PDF]

open access: yes, 2020
Tafazzin (TAZ) is a mitochondrial transacylase that remodels the mitochondrial cardiolipin into its mature form. Through a CRISPR screen, we identified TAZ as necessary for the growth and viability of acute myeloid leukemia (AML) cells.
Seneviratne, Ayesh Kumar
core   +1 more source

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