Download full-text PDF

Source
http://dx.doi.org/10.1097/00008506-199003000-00012DOI Listing

Publication Analysis

Top Keywords

argininosuccinate synthetase
4
synthetase deficiency
4
deficiency reye
4
reye syndrome-like
4
syndrome-like presentation
4
argininosuccinate
1
deficiency
1
reye
1
syndrome-like
1
presentation
1

Similar Publications

Serum proteomic and metabolomic profiling of hepatocellular carcinoma patients co-infected with .

Front Immunol

January 2025

Key Laboratory of Longevity and Aging-related Diseases of Chinese Ministry of Education, Guangxi Medical University, Nanning, China.

Background: () infection is a significant risk factor for hepatocellular carcinoma (HCC), yet its underlying mechanisms remain poorly understood. This study aimed to investigate the impact of infection on the serum proteomic and metabolomic profiling of HCC patients, focusing on the potential mechanisms.

Method: A retrospective clinical analysis was conducted on 1121 HCC patients, comparing those with and without infection.

View Article and Find Full Text PDF

Background/aims: Hepatocellular carcinoma (HCC) is a malignant cancer with an increasing incidence worldwide. Although numerous efforts have been made to identify effective therapies for HCC, current strategies have limitations. We present a new approach for targeting L-arginine and argininosuccinate synthetase 1 (ASS1).

View Article and Find Full Text PDF

[Tandem mass spectrometry screening and genetic analysis of neonates with Urea cycle disorders].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi

January 2025

Department of Medical Genetics and Prenatal Diagnosis, Xuzhou Maternity and Child Health Care Hospital, Xuzhou, Jiangsu 221009, China.

Objective: To explore the results of four types of Urea cycle disorders (UCDs) in newborns from the Xuzhou region, assess the efficacy of newborn screening by tandem mass spectrometry (MS/MS), and analyze their genetic characteristics.

Methods: A retrospective analysis was performed using tandem mass spectrometry to screen for inherited metabolic disorders in 691 712 newborns at the Maternal and Child Health Care Hospital of Xuzhou from November 2015 to December 2023. Ten children (cases 1-10) were diagnosed with Ornithine transcarbamylase deficiency (OTCD), Carbamoylphosphate synthase 1 deficiency (CPS1D), Arginase deficiency (ARGD), and Argininosuccinate synthase deficiency (ASSD) based on MS/MS and genetic testing.

View Article and Find Full Text PDF

Advances in the impact of ASS1 dysregulation on metabolic reprogramming of tumor cells.

Cell Signal

January 2025

Department of Biochemistry and Molecular Biology, School of Basic Medical Sciences, Kunming Medical University, Yunnan, Kunming 650500, PR China. Electronic address:

ASS1(argininosuccinate synthase 1) is a rate-limiting enzyme in the urea cycle, catalyzing the synthesis of argininosuccinate from citrulline and aspartate to ultimately produce arginine and support cellular metabolism. Increasing evidence suggests that ASS1 is commonly dysregulated in the tumor microenvironment, promoting tumor cell metastasis and infiltration. With a deeper understanding of tumor metabolic reprogramming in recent years, the impact of ASS1 dysregulation on abnormal tumor metabolism has attracted growing interest among researchers.

View Article and Find Full Text PDF

Overcoming resistance to arginine deprivation therapy using GC7 in pleural mesothelioma.

iScience

January 2025

Centre for Cancer Cell & Molecular Biology, Barts Cancer Institute, Queen Mary University of London, Charterhouse Square, London EC1M 6BQ, UK.

Pleural mesothelioma is a highly chemotherapy-resistant cancer. Approximately 50% of mesotheliomas do not express argininosuccinate synthetase 1 (ASS1), the rate-limiting enzyme in arginine biosynthesis, making arginine depletion with pegylated arginine deiminase (ADI-PEG20) an attractive therapeutic strategy. We investigated whether combinatory treatment composed of ADI-PEG20 and polyamine inhibitors constitutes a promising novel therapeutic strategy to overcome ADI-PEG20 resistance in mesothelioma patients.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!