Results 21 to 30 of about 80,674 (255)

Mutation of zebrafish dihydrolipoamide branched-chain transacylase E2 results in motor dysfunction and models maple syrup urine disease

Disease Models & Mechanisms, 2012
SUMMARY Analysis of zebrafish mutants that demonstrate abnormal locomotive behavior can elucidate the molecular requirements for neural network function and provide new models of human disease.
Timo Friedrich, Aaron M. Lambert, Mark A. Masino, Gerald B. Downes   +3 more
doaj   +1 more source

Setting the Stage for Branched-Chain Amino Acids Use in Neurological Pathologies: Does a Single Oral Dose Provide Hours of Elevated Systemic Levels?

Diseases
Background: Recent studies have demonstrated that branched-chain amino acids are neuroprotective and neurorestorative. Branched-chain amino acid supplements are now being recommended to be taken before contact sports to reduce concussions.
Ezek Mathew, Nathan Jones, McKinley Dews, Dominique Neal, Anders Cohen   +4 more
doaj   +1 more source

The Aspergillus fumigatus dihydroxyacid dehydratase Ilv3A/IlvC is required for full virulence. [PDF]

PLoS ONE, 2012
Dihydroxyacid dehydratase (DHAD) is a key enzyme in the branched-chain amino acid biosynthetic pathway that exists in a variety of organisms, including fungi, plants and bacteria, but not humans.
Jason D Oliver, Sarah J Kaye, Danny Tuckwell, Anna E Johns, Darel A Macdonald, Joanne Livermore, Peter A Warn, Mike Birch, Michael J Bromley   +8 more
doaj   +1 more source

A Classic Case of Maple Syrup Urine Disease and a Novel Mutation in the BCKDHA Gene [PDF]

Iranian Journal of Neonatology, 2017
Background: Maple syrup urine disease (MSUD) is an inherited branched-chain amino acid metabolic disorder caused by the deficiency in the branched-chain alpha-keto acid dehydrogenase (BCKD) complex.
Alieh Mirzaee, Narges Pishva, Zohreh Karamizadeh, Jurgen Kohlhase, Shahnaz Purarian, Fariba Hemmati, Mostajab Razavi, Shiva Nasirabadi   +7 more
doaj   +1 more source

Isolated branched-chain amino acid intake and muscle protein synthesis in humans: a biochemical review

Einstein (São Paulo)
Alongside a proper diet, ergogenic aids with potential direct and/or indirect physical performance enhancing effects are sought after for improved adaptation to physical training.
Carina de Sousa Santos, Fabrício Expedito Lopes Nascimento   +1 more
doaj   +1 more source

The ubiquitin‐proteasome system and autophagy as guardians of the cellular proteome

FEBS Letters, EarlyView.
This Perspective covers the three principles governing the crosstalk between the ubiquitin‐proteasome system and autophagy in cellular proteostasis: (1) a shared ubiquitin code routing substrates via shuttle factors or autophagy receptors; (2) spatial compartmentalization into phase‐separated degradation hubs and organelle‐specific modules (exemplified
Ivan Dikic
wiley   +1 more source

Decoding the nexus: branched-chain amino acids and their connection with sleep, circadian rhythms, and cardiometabolic health

Neural Regeneration Research
The sleep-wake cycle stands as an integrative process essential for sustaining optimal brain function and, either directly or indirectly, overall body health, encompassing metabolic and cardiovascular well-being.
Hui Li, Laurent Seugnet
doaj   +1 more source

Neutrophils as a source of branched-chain, aromatic and positively charged free amino acids

Cell Adhesion & Migration, 2019
Neutrophils release branched-chain (valine, isoleucine, leucine), aromatic (tyrosine, phenylalanine) and positively charged free amino acids (arginine, ornithine, lysine, hydroxylysine, histidine) when adhere and spread onto fibronectin.
Svetlana I. Galkina, Natalia V. Fedorova, Alexander L. Ksenofontov, Vladimir I. Stadnichuk, Ludmila A. Baratova, Galina F. Sud’Ina   +5 more
doaj   +1 more source

Microbiome−host proteostasis crosstalk—An emerging perspective on mechanisms and interventions toward healthy longevity

FEBS Letters, EarlyView.
Proteostasis and the gut microbiota play a key role in shaping host physiology. Microbiota‐derived metabolites, vitamins, and RNA modulate host proteostasis. Findings from model systems, including C. elegans, indicate microbes can either stabilize or disrupt host proteostasis.
Abhishek Anil Dubey, Maria Ermolaeva
wiley   +1 more source

Production and characterization of murine models of classic and intermediate maple syrup urine disease

BMC Medical Genetics, 2006
Background Maple Syrup Urine Disease (MSUD) is an inborn error of metabolism caused by a deficiency of branched-chain keto acid dehydrogenase. MSUD has several clinical phenotypes depending on the degree of enzyme deficiency.
Watkins Simon, Ferguson Carolyn, Skvorak Kristen, Homanics Gregg E, Paul Harbhajan S   +4 more
doaj   +1 more source