Results 121 to 130 of about 104,062 (224)

The spectrum of phenylalanine hydroxylase variants and genotype-phenotype correlation in phenylketonuria patients in Gansu, China. [PDF]

Hum Genomics, 2023
Zhang C, Zhang P, Yan Y, Zhou B, Wang Y, Tian X, Hao S, Ma P, Zheng L, Zhang Q, Hui L, Wang Y, Cao Z, Ma X.   +13 more
europepmc   +1 more source

Re‐evaluation of salt of aspartame‐acesulfame (E 962) as food additive

EFSA Journal, Volume 24, Issue 9, September 2026.
Abstract The present opinion deals with the re‐evaluation of salt of aspartame‐acesulfame (E 962) as a food additive. The Panel considered that upon ingestion, E 962 dissociates into aspartame and acesulfame ions which correspond to those of the authorised sweeteners aspartame (E 951) and acesulfame K (E 950). The Panel therefore assessed the safety of
EFSA Panel on Food Additives and Flavourings (FAF), Laurence Castle, Monica Andreassen, Gabriele Aquilina, Maria Lourdes Bastos, Polly Boon, Biagio Fallico, Rex FitzGerald, Maria Jose Frutos Fernandez, Bettina Grasl‐Kraupp, Ursula Gundert‐Remy, Rainer Gürtler, Eric Houdeau, Marcin Kurek, Henriqueta Louro, Patricia Morales, Sabina Passamonti, Ellen Bruzell, Cristina Fortes, Kevin James Chipman, Riccardo Crebelli, Peter Fürst, Jean‐Charles Leblanc, Evangelia Ntzani, Andrea Terron, Matthew Wright, Stefania Barmaz, Costanza Bonnici, Consuelo Civitella, Elena Mazzoli, Josef Daniel Rasinger, Ana Rincon, Camilla Smeraldi, Alexandra Tard, Federica Lodi   +34 more
wiley   +1 more source

Hepatocyte Transplantation Using the Domino Concept in a Child with Tetrabiopterin Nonresponsive Phenylketonuria

Cell Transplantation, 2012
Phenylketonuria is a metabolic disease caused by phenylalanine hydroxylase deficiency. Treatment is based on a strict natural protein-restricted diet that is associated with the risk of malnutrition and severe psychosocial burden.
X. Stéphenne, F. G. Debray, F. Smets, N. Jazouli, G. Sana, T. Tondreau, R. Menten, P. Goffette, F. Boemer, R. Schoos, S. W. Gersting, M. Najimi, A. C. Muntau, P. Goyens, E. M. Sokal M.D., Ph.D.   +14 more
doaj   +1 more source

Screening and mutation analysis of phenylalanine hydroxylase deficiency in newborns from Jiangxi province. [PDF]

Front Genet, 2023
Zeng B, Lu Q, Chen S, Guan H, Xu X, Zou Y, Wang F, Huang S, Liu Y, Yang B.   +9 more
europepmc   +1 more source

Activation of Phenylalanine Hydroxylase by Phenylalanine Does Not Require Binding in the Active Site

, 2015
Phenylalanine hydroxylase (PheH), a liver enzyme that catalyzes the hydroxylation of excess phenylalanine in the diet to tyrosine, is activated by phenylalanine. The lack of activity at low levels of phenylalanine has been attributed to the N-terminus of
Kenneth M. Roberts (1340985), Cynthia S. Hinck (1346817), Paul F. Fitzpatrick (1340982), Crystal A. Khan (1346820)   +3 more
core   +1 more source

Structural Studies of the Catalytic and Regulatory Mechanisms of Phenylalanine Hydroxylase [PDF]

, 2010
The catalytic and regulatory mechanisms of phenylalanine hydroxylase were investigated by structural studies of in this research. Phenylalanine hydroxylase (PheH) hydroxylates phenylalanine to produce tyrosine using tetrahydrobiopterin (BH4) and oxygen ...
Li, Jun
core   +1 more source

In Silico Structural Protein Evaluation of the Phenylalanine Hydroxylase p.(Tyr77His) Variant Associated with Benign Hyperphenylalaninemia as Identified through Mexican Newborn Screening. [PDF]

Children (Basel), 2023
Vela-Amieva M, Alcántara-Ortigoza MA, González-Del Angel A, Ibarra-González I, Fernández-Hernández L, Guillén-López S, López-Mejía L, Fernández-Lainez C.   +7 more
europepmc   +1 more source

Comparative transcriptomic and metabolomic analysis reveals mechanisms of selenium-regulated anthocyanin synthesis in waxy maize (Zea mays L.)

Frontiers in Plant Science
Anthocyanins in maize (Zea mays L.) kernels determine the plant’s color and can enhance its resistance. Selenium (Se) significantly impacts plant growth, development, and secondary metabolic regulation.
Guangyu Guo, Guangyu Guo, Yufeng Wang, Baoku Zhang, Haoran Yu, Liang Li, Guanglu Cao, Baicui Chen, Chengxin Li, Fanshan Bu, Song Teng, Qingtao Yu, Mingbo Gao, Baiwen Jiang, Kejun Yang   +14 more
doaj   +1 more source

Deubiquitinase USP19 extends the residual enzymatic activity of phenylalanine hydroxylase variants. [PDF]

Sci Rep, 2022
Sarodaya N, Tyagi A, Kim HJ, Kang JS, Singh V, Hong SH, Kim WJ, Kim KS, Ramakrishna S.   +8 more
europepmc   +1 more source

Two apparent molecular weight forms of human and monkey phenylalanine hydroxylase are due to phosphorylation

, 1984
Two-dimensional polyacrylamide gel analyses of purified human and monkey liver phenylalanine hydroxylase reveal that the enzyme consists of two different apparent molecular weight forms of polypeptide, designated H (M(r) = 50,000) and L (M(r) = 49,000 ...
BE Kemp (11862284), RG Cotton (14528132), WJ McAdam (14574962), Julian Mercer (13076475), SC Smith (11760032)   +4 more
core   +1 more source