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phenylalanine/atrophy

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Neurological deterioration in young adults with phenylketonuria.

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7 patients with phenylketonuria who developed neurological disability in adolescence or early adult life are described. 4 had been diagnosed by routine neonatal screening and started a low phenylalanine diet in infancy. 3 were diagnosed in early childhood because of developmental delay, and then
Biogenic amines synthesis in phenylketonuria (PKU) patients with high phenylalanine (Phe) concentration is thought to be impaired due to inhibition of tyrosine and tryptophan hydroxylases and competition with amino acids at the blood-brain barrier. Dopamine and serotonin deficits might explain brain
Citrus fruit is prone to develop peel pitting during development and storage, which greatly decreases its fresh market value because of the deterioration of the peel. In the present study, we have examined the effect of different temperatures (15 degrees C and 4 degrees C), waxing and mechanical
Objective: This study aimed to assess the association between synovial fluid (SF) metabolites and magnetic resonance imaging (MRI) measurements of cartilage biochemical composition to identify potential SF biomarkers for detecting the early onset of cartilage
The morphologic effects of hyperphenylalaninemia induced by treatment with para-chlorophenylalanine (PCP) plus phenylalanine on optic nerve were studied in developing F344 rats. PCP and phenylalanine were infected daily between days 5 and 20 days of life. At 20 days optic nerve of treated animals,
The Maillard reaction end-products (MRPs) and intermediate (MRI, N-(1-deoxy-α-D-xylulos-1-yl)-phenylalanine) derived from xylose (Xyl) and phenylalanine (Phe) model system were synthesized in an aqueous medium, and their antioxidant activity was evaluated. Both the MRPs and
There is a statistically significant difference in the IQ's of PKU and histidinemia parents. The difference is due entirely to the verbal part of the Hamburg-Wechsler test. There is no significant difference in performance. The heterozygous state of histidinemia does not seem to bear an intellectual

Treatment of phenylalanine hydroxylase deficiency.

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In phenylalanine hydroxylase deficiency detected by screening treatment in early life, both age at start of treatment and phenylalanine control during treatment are the major determinants of eventual psychological status. The influence of phenylalanine control declines with age but executive
It has been shown that BCAAs, especially leucine, regulate skeletal muscle protein metabolism. However, it remains unclear how BCAAs regulate muscle protein metabolism and lead to anabolism in vivo. We examined muscle protein synthesis rate and breakdown rate simultaneously during BCAA infusion in
OBJECTIVE The treatment of phenylketonuria (PKU) in children and adults has been difficult because of erosion of dietary adherence, leading to poor school performance, impairment of executive functioning, loss of IQ, and deterioration of white matter in the brain. Mutant PKU mice produced by
We present the results of a theoretical study on dynamic Stark-induced coherent π-electron rotations in a chiral aromatic ring molecule. This is an extension of our previous papers, which have been published in Mineo , H. [ Phys. Chem. Chem. Phys. 2016 , 18 , 26786 - 26795 ] and Mineo , H. [
OBJECTIVE To observe the effect of Shenshuai Yingyang Capsule (SSYYJN) in ameliorating muscle atrophy in rats with chronic renal failure (CRF) and explore the role of Wnt7a-Akt/mTOR signal pathway in mediating this effect. METHODS Male rats were randomly assigned to 5/6 nephrectomy group and
Background: Unavoidable periods of disuse lead to muscle atrophy and functional decline. Preventing such declines can reduce the risk of re-injury and improve recovery of normal physiological functioning.
Unregulated disinfection byproducts (DBPs), including nitrogenous disinfection byproducts (N-DBPs), originating from chlorination of the precursor amino acid phenylalanine in aqueous systems, were identified in laboratory reactions and distributed tap. The major N-DBP identified was
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