Results 41 to 50 of about 888 (137)

Immunohistochemical localization of aspartoacylase in the rat central nervous system [PDF]

Journal of Comparative Neurology, 2004
AbstractAspartoacylase (ASPA; EC 3.5.1.15) catalyzes deacetylation of N‐acetylaspartate (NAA) to generate free acetate in the central nervous system (CNS). Mutations in the gene coding ASPA cause Canavan disease (CD), an autosomal recessive neurodegenerative disease that results in death before 10 years of age.
Chikkathur N, Madhavarao, John R, Moffett, Roger A, Moore, Ronald E, Viola, M A Aryan, Namboodiri, David M, Jacobowitz   +5 more
openaire   +2 more sources

Metabolic signatures and a diagnostic model for citrin deficiency based on urinary organic acids

Clinical and Translational Medicine, Volume 15, Issue 9, September 2025.
Urinary organic acid profiles in NICCD show enrichment in energy and amino acid pathways. The random forest model differentiates NICCD from non‐specific metabolic abnormalities, both with elevated 4‐hydroxyphenyllactic acid and 4‐hydroxyphenylpyruvic acid, which may cause misdiagnosis. Model explanation and web application help physicians make clinical
Peiyao Wang, Peichun Chen, Xinjie Yang, Ziyan Cen, Yu Zhang, Qimin He, Benqing Wu, Xinwen Huang   +7 more
wiley   +1 more source

Bovine Endometrium Drives and Responds to Divergence of In Vitro Produced Conceptus Biochemistry

The FASEB Journal, Volume 39, Issue 16, 31 August 2025.
IVD versus IVP bovine conceptus‐endometrial microenvironment. Compared to the in vivo derived (IVD) bovine conceptus, the in vitro produced (IVP) conceptus has a reduced number of transcribed genes, mRNA abundance for genes transcribed and increased expression of inflammatory mediators in response to the endometrium.
Katheryn D. Peterson, Mary A. Oliver, Trevor F. Freeman, Shankar P. Poudel, Susanta K. Behura, David Kakhniashvili, Daniel L. Johnson, Rebecca R. Payton, Tulio M. Prado, Lew G. Strickland, J. Lannett Edwards, Elizabeth A. Shepherd, Jonathan E. Beever, Thomas E. Spencer, Daniel J. Mathew   +14 more
wiley   +1 more source

Valdiazen Derivatives for Chemoproteomic Studies in Burkholderia cenocepacia H111

ChemBioChem, Volume 26, Issue 11, June 3, 2025.
Valdiazen is a quorum‐sensing signal isolated from B. c. H111. Using diazeniumdiolate chemical probes, several valdiazen‐binding proteins are identified, many of which are involved in structural and metabolic processes. This study offers valuable chemical tools and advances our understanding of the interactome associated with diazeniumdiolate signaling
Jiajun Ren, Anugraha Mathew, María Rodríguez‐García, Colin C. Regli, Olivier Blacque, Bernhard Spingler, Simon Sieber, Leo Eberl, Karl Gademann   +8 more
wiley   +1 more source

Regulación de la utilización del aspartato por los astrocitos durante la prelactancia

Universitas Scientiarum, 2009
Regulation of the use of aspartate by the astrocytes during the presuckling period. During the perinatal period the concentration ofN-acetyl-L-aspartate (NAA) produced by neurons increases.
Cecilia Espíndola, Ludis Morales, María Juliana Salamanca Ortiz, Jairo Alfonso Tovar-Franco   +3 more
doaj  

Human induced pluripotent stem cell line (SDQLCHi064-A) derived from a patient with Canavan disease carrying c.556_559dup GTTC and c.919delA mutations in the ASPA gene

Stem Cell Research
Canavan disease (CD, OMIM# 271900) is an autosomal recessive neurodegenerative disorder caused by homozygous or compound heterozygous mutations in ASPA gene, which result in catalytic deficiency of the aspartoacylase enzyme and the accumulation of N ...
Ning Liu, Yongsheng Ge, Yanan Yang, Fen Zhao, Yuqiang Lv, Zilong Li, Rui Dong, Yi Liu, Zhongtao Gai   +8 more
doaj   +1 more source

The contribution of extracellular RNA and its derived biomaterials in disease management

BMEMat, Volume 3, Issue 2, June 2025.
The implications of exRNA's biological function and the structural uniqueness of RNA help establish a close connection between RNA, material, and modern medicine. Abstract The RNA found in the circular system is known as extracellular RNA (exRNA). This kind of RNA has been found to play a biological role similar to that of a messenger. They can be used
Yu Wei, Zhangyi Wang, Zixuan Qin, Qianqian Wan, Yutao Li, Franklin R. Tay, Chenyu Wang, Tong Zhang, Lina Niu   +8 more
wiley   +1 more source

Structure of aspartoacylase, the brain enzyme impaired in Canavan disease [PDF]

Proceedings of the National Academy of Sciences, 2007
Aspartoacylase catalyzes hydrolysis of N -acetyl- l -aspartate to aspartate and acetate in the vertebrate brain. Deficiency in this activity leads to spongiform degeneration of the white matter of the brain and is the established cause of Canavan disease, a fatal progressive leukodystrophy ...
Eduard, Bitto, Craig A, Bingman, Gary E, Wesenberg, Jason G, McCoy, George N, Phillips   +4 more
openaire   +2 more sources

Developmental maturation and regional heterogeneity but no sexual dimorphism of the murine CNS myelin proteome

Glia, Volume 73, Issue 1, Page 38-56, January 2025.
Main Points We analyze myelin purified from mouse CNS tissue by quantitative proteomics. The protein composition of myelin matures between P18 and P75. Regional heterogeneity but no sex‐dependent divergence of the CNS myelin proteome. Abstract The molecules that constitute myelin are critical for the integrity of axon/myelin‐units and thus speed and ...
Sophie B. Siems, Vasiliki‐Ilya Gargareta, Leonie C. Schadt, Vinicius Daguano Gastaldi, Ramona B. Jung, Lars Piepkorn, Patrizia Casaccia, Ting Sun, Olaf Jahn, Hauke B. Werner   +9 more
wiley   +1 more source

Unraveling the role of oligodendrocytes and myelin in pain

Journal of Neurochemistry, Volume 169, Issue 1, January 2025.
This review includes 12 studies focusing on neuropathic pain resulting from peripheral nerve or spinal cord injuries, chemotherapy, and HIV. The studies indicate that reduced oligodendrocyte numbers and decreased myelin thickness may play a crucial role in the development or maintenance of pain. Additionally, an increase in OPCs could aid in repair, as
Woojin Kim, María Cecilia Angulo
wiley   +1 more source