Results 51 to 60 of about 2,890 (187)

The heteromultimeric debranching enzyme involved in starch synthesis in Arabidopsis requires both isoamylase1 and isoamylase2 subunits for complex stability and activity.

PLoS ONE, 2013
Isoamylase-type debranching enzymes (ISAs) play an important role in determining starch structure. Amylopectin - a branched polymer of glucose - is the major component of starch granules and its architecture underlies the semi-crystalline nature of ...
Maria Sundberg, Barbara Pfister, Daniel Fulton, Sylvain Bischof, Thierry Delatte, Simona Eicke, Michaela Stettler, Steven M Smith, Sebastian Streb, Samuel C Zeeman   +9 more
doaj   +1 more source

The Management and Clinical Outcomes of Pregnancy in a Female With Glycogen Storage Disease Type IIIA Caused by Rare Variant

JIMD Reports, Volume 66, Issue 4, July 2025.
ABSTRACT Glycogen storage disease type III (GSD III) is an inborn error of carbohydrate metabolism with an autosomal recessive inheritance pattern. Phenotypically, patients can manifest a broad clinical spectrum. Most patients affected with GSD IIIA (85%) have a non‐functional GDE enzyme primarily affecting the liver and cardiac/skeletal muscle (Type ...
Nuria Puente‐Ruiz, Saru Palaniappan, Alison Woodall, Robert Cooper, Allyson Terry, Andrew Oldham, Abigail Rousseau, Christopher Campbell, Pradeep Vasudevan, Karolina M. Stepien   +9 more
wiley   +1 more source

The Autophagic Activator GHF-201 Can Alleviate Pathology in a Mouse Model and in Patient Fibroblasts of Type III Glycogenosis

Biomolecules
Glycogen storage disease type III (GSDIII) is a hereditary glycogenosis caused by deficiency of the glycogen debranching enzyme (GDE), an enzyme, encoded by Agl, enabling glycogen degradation by catalyzing alpha-1,4-oligosaccharide side chain transfer ...
Kumudesh Mishra, Sahar Sweetat, Saja Baraghithy, Uri Sprecher, Monzer Marisat, Sultan Bastu, Hava Glickstein, Joseph Tam, Hanna Rosenmann, Miguel Weil, Edoardo Malfatti, Or Kakhlon   +11 more
doaj   +1 more source

Ecogenomics and limnological dynamics of a new Thiocapsa species blooming in the whole water column of a karstic lake

Limnology and Oceanography, Volume 70, Issue 7, Page 1849-1866, July 2025.
Abstract Purple sulfur bacteria (PSB) of the family Chromatiaceae (Gammaproteobacteria) can perform chemo‐ and photo‐lithoautotrophy (through anoxygenic photosynthesis) in anoxic layers of freshwater stratified (including meromictic) lakes. This group has been extensively studied via physiological and ecological approaches, albeit their genomics has ...
Pedro J. Cabello‐Yeves, Antonio Picazo, Carlos Rochera, Daniel Morant, Eduardo Vicente, David J. Scanlan, Richard J. Puxty, Antonio Camacho   +7 more
wiley   +1 more source

Phase Separation Regulates Metabolism, Mitochondria, and Diseases

MedComm, Volume 6, Issue 7, July 2025.
Mitochondrion‐related liquid–liquid phase separation. (1) Phase separation mediates the self‐assembly of mitochondrial nucleoids (mt‐nucleoids). (2) Phase segregation mediates the formation of mitochondrial RNA granules (MRGs). (3) Phase separation mediates the formation of mitochondrial degradosomes.
Chuan Gao, Peng Ding, Changqing Zhang, Junjie Gao   +3 more
wiley   +1 more source

Phenylketonuria and glycogen storage disease type III in sibs of one family

The Turkish Journal of Pediatrics, 2002
Hyperphenylalaninemia result from a block in the conversion of phenylalanine into tyrosine due to a defect in either the enzyme phenylalanine hydroxylase (98% of subjects) or in the metabolism of the cofactor tetrahydrobiopterin.
Tuncay Yilmazer, Albert H Van Gennip, Nico G G M Abeling, Imran Ozalp, Turgay Coşkun, Henk D Bakker   +5 more
doaj  

A founder splice site mutation underlies glycogen storage disease type 3 in consanguineous Saudi families

Annals of Saudi Medicine, 2014
BACKGROUND AND OBJECTIVES: Glycogen storage disease type 3 (GSD III) is an autosomal recessive disorder caused by genetic mutations in the gene AGL. AGL encodes amylo-α-1, 6-glucosidase, 4-α-glucanotransferase, a glycogen debranching enzyme.
Sulman Basit, Omhani Malibari, Alia Mahmood Al Balwi, Firoz Abdusamad, Feras Abu Ismail   +4 more
doaj   +1 more source

Leveraging core enzyme structures for microbiota targeted functional regulation: Urease as an example

iMeta, Volume 4, Issue 3, June 2025.
We present an innovative methodology that integrates core enzyme identification, protein structural characterization, regulator virtual screening, and functional validation to achieve precise microbiome functional regulation. As a proof of concept, we focused on the regulation of urea decomposition by the rumen microbiota in ruminants. This methodology
Shengguo Zhao, Huiyue Zhong, Yue He, Xiaojiao Li, Li Zhu, Zhanbo Xiong, Xiaoyin Zhang, Nan Zheng, Diego P. Morgavi, Jiaqi Wang   +9 more
wiley   +1 more source

A novel genetic model provides a unique perspective on the relationship between postexercise glycogen concentration and increases in the abundance of key metabolic proteins after acute exercise.

PLoS ONE
Some acute exercise effects are influenced by postexercise (PEX) diet, and these diet-effects are attributed to differential glycogen resynthesis. However, this idea is challenging to test rigorously. Therefore, we devised a novel genetic model to modify
Seong Eun Kwak, Amy Zheng, Edward B Arias, Haiyan Wang, Xiufang Pan, Yongping Yue, Dongsheng Duan, Gregory D Cartee   +7 more
doaj   +1 more source

Grain under pressure: Harnessing biochemical pathways to beat drought and heat in wheat

The Plant Journal, Volume 122, Issue 6, June 2025.
SUMMARY Erratic climate patterns represent a remarkable challenge to global food security, particularly affecting staple cereal crops of which wheat (Triticum aestivum) plays a critical role in annual agricultural production globally. It has been shown that over the last four decades, wheat cultivation has faced an escalating vulnerability to a variety
Itsuhiro Ko, Tyler Chapman, Taras Nazarov, Ruth Uwugiaren, Andrei Smertenko, Niharika Nonavinakere Chandrakanth, Dylan Oates   +6 more
wiley   +1 more source