Results 131 to 140 of about 3,274 (204)

CsSSCD1-mediated tyrosine catabolism redirects nitrogen flux to regulate theanine accumulation and drought adaptation in tea plants

Industrial Crops and Products
The tyrosine degradation pathway maintains amino acid homeostasis in plants, yet its function in tea (Camellia sinensis) remains unclear. Here, we identified CsSSCD1, encoding fumarylacetoacetate hydrolase (FAH), as a key enzyme linking tyrosine ...
Dayu Huang, Dexiang Yao, Wei Xu, Cui Lu, Tong Li, Litang Lu, Yihe Jiang   +6 more
doaj   +1 more source

Transcriptome analysis reveals manifold mechanisms of cyst development in ADPKD [PDF]

, 2016
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) causes progressive loss of renal function in adults as a consequence of the accumulation of cysts. ADPKD is the most common genetic cause of end-stage renal disease. Mutations in polycystin-
Bacallao, Robert L., Boedigheimer, Michael, Clendenon, Sherry G., Damore, Michael, de Almeida, Rita M. C., Glazier, James A., Harris, Peter C., Herbert, Britney-Shea, Richards, William G., Rossetti, Sandro, Wandinger-Ness, Angela, Ward, Heather H., Xu, Wei Min   +12 more
core   +2 more sources

Perinatal Gene Transfer to the Liver [PDF]

, 2011
The liver acts as a host to many functions hence raising the possibility that any one may be compromised by a single gene defect. Inherited or de novo mutations in these genes may result in relatively mild diseases or be so devastating that death within
Buckley, SM, Chan, JK, Howe, SJ, McKay, TR, Rahim, AA, Waddington, SN, Ward, NJ   +6 more
core   +1 more source

CRISPR/Cas9-Mediated Knockout of BnaFAH Enhanced Brassica napus Resistance to Plutella xylostella Under a 2-Day Short-Day Photoperiod

Horticulturae
The diamondback moth (Plutella xylostella) severely threatens global oilseed rape (Brassica napus L.) production. This study demonstrates that CRISPR/Cas9-mediated knockout of two homologous BnaFAH, involved in tyrosine degradation, confers enhanced ...
Tiantian Zhi, Zhou Zhou, Chen Shi, Meiqiong Xie, Gang Chen, Cui Lu   +5 more
doaj   +1 more source

Mitochondrial enzyme FAHD1 reduces ROS in osteosarcoma

Scientific Reports
This study investigated the impact of overexpressing the mitochondrial enzyme Fumarylacetoacetate hydrolase domain-containing protein 1 (FAHD1) in human osteosarcoma epithelial cells (U2OS) in vitro.
Anne Heberle, Elia Cappuccio, Andreas Andric, Tatjana Kuen, Anna Simonini, Alexander K. H. Weiss   +5 more
doaj   +1 more source

Progress in Gene Therapy for Hereditary Tyrosinemia Type 1

Pharmaceutics
Hereditary Tyrosinemia Type-1 (HT1), an inherited error of metabolism caused by a mutation in the fumarylacetoacetate hydrolase gene, is associated with liver disease, severe morbidity, and early mortality.
Helen Thomas, Robert C. Carlisle
doaj   +1 more source

The inducers 1,3-diaminopropane and spermidine cause the reprogramming of metabolism in Penicillium chrysogenum, leading to multiple vesicles and penicillin overproduction [PDF]

, 2013
In this article we studied the differential protein abundance of Penicillium chrysogenum in response to either 1,3-diaminopropane (1,3-DAP) or spermidine, which behave as inducers of the penicillin production process.
Barreiro, Carlos., García-Estrada, Carlos., Jami, Mohammad-Saeid., Martín, Juan-Francisco., Martín-González, Jorge.   +4 more
core  

Loss of fumarylacetoacetate hydrolase is responsible for the neonatal hepatic dysfunction phenotype of lethal albino mice. [PDF]

, 1993
Markus Grompe, M al-Dhalimy, Milton J. Finegold, C N Ou, T. Burlingame, N. G. Kennaway, Philippe Soriano   +6 more
openalex   +1 more source

A Lithuanian Case of Tyrosinemia Type 1 with a Literature Review: A Rare Cause of Acute Liver Failure in Childhood

Medicina
Hereditary type 1 tyrosinemia (HT1) is a rare inherited autosomal recessive disorder of tyrosine metabolism, characterized by progressive liver damage, dysfunction of kidney tubules, and neurological crises.
Rūta Rokaitė, Agnė Čibirkaitė, Vykinta Zeleckytė, Gabija Lazdinytė, Mindaugas Dženkaitis   +4 more
doaj   +1 more source

Point mutations in the Murine Fah gene : animal models for the human genetic disorder hereditary tyrosinemia type 1 [PDF]

, 1997
Hereditary tyrosinemia type 1 (HT1) is a severe autosomal recessive metabolic disease, associated with point mutations in the human fumarylacetoacetate hydrolase (FAH) gene that cause disruption of tyrosine catabolism.
Aponte, Jennifer Lynn
core   +1 more source