Results 1 to 10 of about 1,058 (113)

Moco Carrier and Binding Proteins

Molecules, 2022
The molybdenum cofactor (Moco) is the active site prosthetic group found in numerous vitally important enzymes (Mo-enzymes), which predominantly catalyze 2 electron transfer reactions. Moco is synthesized by an evolutionary old and highly conserved multi-
Tobias Kruse
exaly   +3 more sources

The History of the Molybdenum Cofactor—A Personal View

Molecules, 2022
The transition element molybdenum (Mo) is an essential micronutrient for plants, animals, and microorganisms, where it forms part of the active center of Mo enzymes.
Ralf R Mendel
exaly   +3 more sources

The Final Step in Molybdenum Cofactor Biosynthesis—A Historical View [PDF]

Molecules
Molybdenum (Mo) is an essential micronutrient across all kingdoms of life, where it functions as a key component of the active centers of molybdenum-dependent enzymes.
Ralf R. Mendel, Kevin D. Oliphant
doaj   +2 more sources

Impaired gephyrin G-domain trimerization and phase separation in a patient with developmental epileptic encephalopathy [PDF]

EMBO Molecular Medicine
Epilepsy, a common neurological disorder is frequently linked to genetic variants in synaptic proteins. Here, we describe a de novo pathogenic missense variant in the gephyrin G-domain (G134R) identified in an individual with developmental delay ...
Emanuel H W Bruckisch, Marcelo de Melo Aragão, Thais dos Santos Rohde, Ann-Kathrin Huber, Mateus de Oliveira Torres, Günter Schwarz, Filip Liebsch   +6 more
doaj   +2 more sources

Phylogenetic based dissection of eukaryotic Mo-insertase functionality: From mechanism to complex assembly. [PDF]

PLoS ONE
Molybdenum cofactor (Moco) biosynthesis is vitally important for all organisms, yet the domain organization of the eukaryotic molybdenum insertase (Mo-insertase) remains enigmatic.
Tim Julian Schmidt, Ahmed H Hassan, Boas Pucker, Tobias Kruse   +3 more
doaj   +2 more sources

Novel pathogenic variant in a mild case of type B molybdenum cofactor deficiency: case report and literature review [PDF]

BMC Medical Genomics
Background Molybdenum cofactor deficiency (MoCD) is a rare metabolic disorder caused by pathogenic variants in the highly conserved biosynthetic pathway of molybdenum cofactor (MoCo), resulting in sulfite intoxication.
Morgan Kinsinger, Jelena Ivanisevic, Divakar S. Mithal   +2 more
doaj   +2 more sources

Evolutionary plasticity and functional repurposing of the essential metabolic enzyme MoeA [PDF]

Communications Biology
MoeA, also known as gephyrin in higher eukaryotes, is an enzyme essential for molybdenum cofactor (Moco) biosynthesis and involved in GABA and GlyR receptor clustering at the synapse in animals.
Daniela Megrian, Mariano Martinez, Pedro M. Alzari, Anne Marie Wehenkel   +3 more
doaj   +2 more sources

Case Report: Compound Heterozygous Variants in MOCS3 Identified in a Chinese Infant With Molybdenum Cofactor Deficiency

Frontiers in Genetics, 2021
Background: The molybdenum cofactor (Moco) deficiency in humans results in the inactivity of molybdenum-dependent enzymes and is caused by pathogenic variants in MOCS1 (Molybdenum cofactor synthesis 1), MOCS2 (Molybdenum cofactor synthesis 2), and GPHN ...
Qi Tian, Yang Cao, Li Shu, Li Shu, Li Shu, Yongjun Chen, Ying Peng, Yaqin Wang, Yuanyuan Chen, Hua Wang, Hua Wang, Xiao Mao, Xiao Mao   +12 more
doaj   +1 more source

Function of Molybdenum Insertases

Molecules, 2022
For most organisms molybdenum is essential for life as it is found in the active site of various vitally important molybdenum dependent enzymes (Mo-enzymes).
Tobias Kruse
doaj   +1 more source

Beyond Moco Biosynthesis―Moonlighting Roles of MoaE and MOCS2

Molecules, 2022
Molybdenum cofactor (Moco) biosynthesis requires iron, copper, and ATP. The Moco-containing enzyme sulfite oxidase catalyzes terminal oxidation in oxidative cysteine catabolism, and another Moco-containing enzyme, xanthine dehydrogenase, functions in ...
Tamaki Suganuma
doaj   +1 more source