Disorders of Valine-Isoleucine Metabolism

Author(s):  
Bruce A. Barshop
1994 ◽  
Vol 40 (10) ◽  
pp. 1879-1883 ◽  
Author(s):  
M J Bennett ◽  
S Powell ◽  
D J Swartling ◽  
K M Gibson

Abstract Tiglyglycine (TG), an intermediate product of the catabolism of isoleucine, is increased in the urine of patients with beta-ketothiolase deficiency or with disorders of propionate metabolism. It is also implicated as a useful diagnostic marker in disorders of the respiratory chain. We present a method for the synthesis of TG and tiglyl[13C, 15N]glycine and the development of a stable isotope dilution mass spectrometric assay for TG. We compare data from controls with that from subjects with beta-ketothiolase deficiency and propionyl-CoA carboxylase deficiency, and with six patients with enzyme-confirmed disorders of the respiratory chain. TG was increased in the urine from all of the patient groups. The increased TG excretion did not persist in one patient with a respiratory chain defect, which suggests that, in some patients, multiple sample analysis may be necessary to identify a respiratory chain defect. This is the first urinary compound to be implicated as a potential marker of disorders of the respiratory chain.


Animals ◽  
2020 ◽  
Vol 10 (11) ◽  
pp. 2125
Author(s):  
Limin Wei ◽  
Bo Zeng ◽  
Siyuan Zhang ◽  
Feng Li ◽  
Fanli Kong ◽  
...  

The gut microbiota coevolve with the host and can be stably transmitted to the offspring. Host genetics plays a crucial role in the composition and abundance of gut microbiota. Inbreeding can cause a decrease of the host’s genetic diversity and the heterozygosity. In this study, we used 16S rRNA gene sequencing to compare the differences of gut microbiota between the Diannan small-ear pig and Banna minipig inbred, aiming to understand the impact of inbreeding on the gut microbiota. Three dominant bacteria (Stenotrophlomonas, Streptococcus, and Lactobacillus) were steadily enriched in both the Diannan small-ear pig and Banna minipig inbred. After inbreeding, the gut microbiota alpha diversity and some potential probiotics (Bifidobacterium, Tricibacter, Ruminocaccae, Christensenellaceae, etc.) were significantly decreased, while the pathogenic Klebsiella bacteria was significantly increased. In addition, the predicted metagenomic analysis (PICRUSt2) indicated that several amino acid metabolisms (‘‘Valine, leucine, and isoleucine metabolism’’, ‘‘Phenylalanine, tyrosine, and tryptophan biosynthesis’’, ‘‘Histidine metabolism’’) were also markedly decreased after the inbreeding. Altogether our data reveal that host inbreeding altered the composition and the predicted function of the gut microbiome, which provides some data for the gut microbiota during inbreeding.


2005 ◽  
Vol 58 (3) ◽  
pp. 488-491 ◽  
Author(s):  
Celia Perez-Cerda ◽  
Judit García-Villoria ◽  
Rob Ofman ◽  
Pedro Ruiz Sala ◽  
Begoña Merinero ◽  
...  

1974 ◽  
Vol 118 (1) ◽  
pp. 103-111 ◽  
Author(s):  
Robert S. Conrad ◽  
Linda K. Massey ◽  
John R. Sokatch

2009 ◽  
Vol 106 (35) ◽  
pp. 14820-14824 ◽  
Author(s):  
S.-Y. Yang ◽  
X.-Y. He ◽  
S. E. Olpin ◽  
V. R. Sutton ◽  
J. McMenamin ◽  
...  

PEDIATRICS ◽  
1972 ◽  
Vol 50 (6) ◽  
pp. 890-895
Author(s):  
James P. Keating ◽  
Ralph D. Feigin ◽  
Stanley M. Tenenbaum ◽  
Richard E. Hillman

A new metabolic defect, clinically mimicking pyloric stenosis, propionic acidemia, and methylmalonic acidemia is described. Although the specific enzymatic defect has not been identified, the infant's response to protein restriction has been excellent. Unfortunately, some evidences of developmental delay persist. Earlier recognition and institution of therapy may, as in allied disorders, be associated with normal physical and intellectual development.


1974 ◽  
Vol 57 (3) ◽  
pp. 269-281 ◽  
Author(s):  
D. Gompertz ◽  
J.M. Saudubray ◽  
C. Charpentier ◽  
K. Bartlett ◽  
P.A. Goodey ◽  
...  

Weed Science ◽  
1985 ◽  
Vol 33 (4) ◽  
pp. 479-483 ◽  
Author(s):  
Paul C. Anderson ◽  
Kenneth A. Hibberd

Effects of the imidazolinone herbicide AC243 997 [2-(5-isopropyl)-5-methyl-4-oxo-2-imidazolin-2-yl) nicotinic acid] on the growth of corn (Zea mays L. ‘Black Mexican Sweet’) cell suspension cultures were characterized. The herbicide caused half maximal growth inhibition at concentrations in the range of 10 nM to 30 nM. Significant reductions in protein synthesis were observed following treatment of cells with 10 μM herbicide. Analysis of free amino acid pool sizes indicated that levels of the biosyn-thetically related amino acids leucine and valine were substantially decreased in cells exposed to 10 μM herbicide. Supplementation of suspension culture growth media with leucine and valine plus isoleucine (1 mM each) reversed growth inhibitory effects of AC243 997 at levels ranging from 10 nM to 1 mM. The results suggest a specific interaction of this imidazolinone herbicide with leucine, valine, and isoleucine metabolism.


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