Holocarboxylase Synthetase Deficiency: A Second Case Report With Neonatal Cholestatic Liver Disease
JIMD Reports, Volume 67, Issue 1, January 2026.ABSTRACT Holocarboxylase synthetase deficiency is an autosomal recessive inborn error of metabolism characterised by life‐threatening metabolic acidosis, ketoacidosis and hyperammonaemia through reduced biotin‐dependent carboxylase activity. We report the presentation of a Polynesian neonate with severe metabolic acidosis secondary to holocarboxylase ...
Sophie Manoy +9 more
wiley +1 more source
Conserved spatial patterning of gene expression in independent lineages of C4 plants
New Phytologist, Volume 249, Issue 1, Page 24-38, January 2026.Summary C4 photosynthesis enhances carbon fixation efficiency by reducing photorespiration through the use of an oxygen‐insensitive carboxylase and spatial separation of photosynthesis between mesophyll and bundle sheath cells. The C4 pathway has evolved independently in > 60 plant lineages, but molecular mechanisms underpinning this convergence remain
Tianshu Sun +4 more
wiley +1 more source
The presence of pyruvate carboxylase in the human brain and its role in the survival of cultured human astrocytes. [PDF]
Physiol Res, 2023 Gondáš E +7 more
europepmc +1 more source
New Phytologist, Volume 249, Issue 2, Page 675-690, January 2026.Summary Photorespiration is a major source of photosynthetic inefficiency in C3 crops. In photorespiration, the oxygenation rather than carboxylation of RuBP by Rubisco triggers an energy‐expensive pathway to recycle inhibitory byproducts and recapture lost carbon, ultimately reducing yields.
Katherine Meacham‐Hensold +3 more
wiley +1 more source
Clinical, biochemical and molecular characterization of 12 patients with pyruvate carboxylase deficiency treated with triheptanoin. [PDF]
Mol Genet Metab, 2023 Lasio MLD +14 more
europepmc +1 more source
Unravelling the Significance of Phosphoenolpyruvate Carboxylase in Phosphate Starvation Responses
Plant, Cell &Environment, Volume 49, Issue 1, Page 177-192, January 2026.ABSTRACT Low phosphate availability is a major concern for agriculture. Plants develop a plethora of responses to improve phosphate acquisition, known as phosphate starvation responses (PSR). Among them, the induction of phosphoenolpyruvate carboxylase (PEPC) has been described in many plants. However, most studies have been conducted in the absence of
Jesús Pérez‐López +10 more
wiley +1 more source
Case Report: Prenatal neurological injury in a neonate with pyruvate carboxylase deficiency type B. [PDF]
Front Endocrinol (Lausanne), 2023 Xue M.
europepmc +1 more source
Metabolite Control Overrides Circadian Regulation of Phosphoenolpyruvate Carboxylase Kinase and CO2 Fixation in Crassulacean Acid Metabolism [PDF]
, 1999 Anne M. Borland +4 more
openalex +1 more source
Advanced Science, Volume 12, Issue 47, December 18, 2025.A genome‐scale model guided the systematic metabolic engineering of Saccharopolyspora spinosa, enabling a 5.5‐fold boost in the green bioinsecticide spinosad to 1.8 g L−1 and establishing an actinomycete framework for complex natural products overproduction.
Shuliu Wang +9 more
wiley +1 more source