Results 81 to 90 of about 20,316 (189)

Determinação dos níveis da carnitina no plasma sua importância no estudo em doentes com doenças neuromusculares.

Acta Médica Portuguesa, 2002
L-carnitine plays a major role in the transport of the long chain fatty acids for the beta-oxidation in the mitochondria. The deficiency of the carnitine is associated by the deficit of the beta-oxidation of the long chain fatty acids and so a different ...
Maria Cíntia Siqueira, Fernando Morgado
doaj   +1 more source

From the cytosol to the inner membrane: biogenesis of the mitochondrial carrier family

Protein Science, Volume 35, Issue 4, April 2026.
Abstract Mitochondrial carrier proteins are essential for cellular physiology as they are active in a wide range of metabolic pathways including production of cellular energy, amino acid synthesis, redox balance and ion homeostasis. The double membrane of mitochondria provides a tightly gated environment through which carrier proteins facilitate the ...
Catherine S. Palmer, Phoebe J. Leeming, Diana Stojanovski   +2 more
wiley   +1 more source

Medium-Chain Acyl-CoA Dehydrogenase Deficiency (MCADD) Newborn Screening in Italy: Five Years’ Experience from a Nationwide Program

International Journal of Neonatal Screening
Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is an autosomal recessive disorder of fatty acid oxidation that can have life-threatening consequences if not promptly treated.
Margherita Ruoppolo, Cristina Cereda, Teresa Giovanniello, Sabrina Malvagia, Sara Boenzi, Francesca Teofoli, on behalf of the SIMMESN Italian Newborn Screening Group, Alberto Burlina   +7 more
doaj   +1 more source

Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts

Journal of Lipid Research, 1998
The relationship between peroxisomal and mitochondrial oxidation of the methyl branched fatty acids, phytanic acid and pristanic acid, was studied in normal and mutant human skin fibroblasts with established enzyme deficiencies.
Nanda M. Verhoeven, Diane S. Roe, Robert M. Kok, Ronald J.A. Wanders, Cornelis Jakobs, Charles R. Roe   +5 more
doaj   +1 more source

The depletion of serine beta‐lactamase‐like protein (LACTB) ameliorates metabolic dysfunction–associated steatotic liver disease by reducing ubiquitin‐mediated degradation of carnitine palmitoyltransferase 2

Diabetes, Obesity and Metabolism, Volume 28, Issue 4, Page 2962-2977, April 2026.
Abstract Aims Metabolic dysfunction–associated steatotic liver disease (MASLD) represents one of the most common chronic liver disorders worldwide, and its incidence continues to rise each year. Serine β‐lactamase‐like protein (LACTB) is a serine protease that plays a crucial role in lipid metabolism and hepatocellular carcinoma, but its function in ...
Wujiang Shi, Xin Liu, Nan Wang, Qianwen Zhang, Jianjun Gao, Canghai Guan, Yapeng Li, Chengru Yang, Shaowu Bi, Xinlei Zou, Xiangyu Zhong   +10 more
wiley   +1 more source

Carnosine and Acyl Carnitines as Metabolic Determinants of Muscle Phenotypic Differences Between Longissimus Dorsi and Triceps Brachii in Hanzhong Sheep

Foods
Muscle traits are critical determinants of meat quality and productivity in sheep, influenced by both breed and anatomical region. Hanzhong sheep, an indigenous Chinese breed, are prized for tender, low-odor meat; yet, the molecular mechanisms underlying
Zhi Li, Miaohua Zheng, Weiwei Li, Jiayi Li, Ling Wang, Shanshan Wang, Hongzhao Lu, Tao Zhang   +7 more
doaj   +1 more source

OCTN2 Activates a Non‐Canonical Carnitine Metabolic Pathway to Promote MASH‐HCC Progression and Immunotherapy Resistance

Advanced Science, Volume 13, Issue 16, 18 March 2026.
In non‐MASH‐HCC, L‐carnitine promotes tumor progression primarily through its classical role in enhancing fatty acid oxidation (FAO). However, in MASH‐HCC, where FAO is markedly suppressed, L‐carnitine shifts from this canonical function to serve instead as an intracellular acetyl group buffer.
Chuqi Xia, Xiao Zhang, Jinze Li, Ning Xu, Sheng Hu, Qiyu Lu, Yuxuan Li, Taifu Xiao, Xu Li, Xue Wang, Kequan Xu, Daoming Liang   +11 more
wiley   +1 more source

Addition of Multi‐Level Technologies to Evaluate Eggs and Embryos and Improve Endometrial Quality Applied for In Vitro Fertilization to Achieve Pregnancy: A Case Study

Clinical Case Reports, Volume 14, Issue 3, March 2026.
This integrated clinical approach optimizes the reproductive cycle in several steps, starting with nutrition (A): restricting carbohydrates and inducing ketosis to reduce inflammation and improve insulin sensitivity, which benefits follicular development.
Tania G. Rojas‐Pérez, Yúvika Reyes‐Albarracín, Ginna Ortiz, Juan José Suárez, Diego Vilchis Reyes, Jacqueline Cordero Oropeza, Dinorah Hernández‐Melchor, Esther López‐Bayghen   +7 more
wiley   +1 more source

Corresponding increase in long-chain acyl-CoA and acylcarnitine after exercise in muscle from VLCAD mice

Journal of Lipid Research, 2009
Long-chain acylcarnitines accumulate in long-chain fatty acid oxidation defects, especially during periods of increased energy demand from fat. To test whether this increase in long-chain acylcarnitines in very long-chain acyl-CoA dehydrogenase (VLCAD−/−)
Frank ter Veld, Sonja Primassin, Lars Hoffmann, Ertan Mayatepek, Ute Spiekerkoetter   +4 more
doaj   +1 more source

The multifaceted roles of the ACSL family in cancer: Metabolic reprogramming, ferroptosis regulation and tumour immune microenvironment remodelling

Clinical and Translational Medicine, Volume 16, Issue 3, March 2026.
The acyl‐CoA synthetase long‐chain (ACSL) family consists of five members, which exhibit a synergistic effect in promoting tumour lipid metabolism for energy supply. Specifically, ACSL3 and ACSL4 have antagonistic effects on the mechanisms related to ferroptosis in tumour cells.
Haocai Li, Weijian Wang, Juncheng Zhan, Yuxiang Xiao, Xiaoping Chen, Chen Su, Peng Zhu   +6 more
wiley   +1 more source