Identity of mitochondrial and cytosolic glycerate kinases in rat liver and regulation of their intracellular localization by dietary protein

Kitagawa, Yasuo; Katayama, Hirokazu; Sugimoto, Etsuro

doi:10.1016/0304-4165(79)90389-1

Cited by 12 publications

(7 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The latter reaction is of little or no importance in the human (10). Evidence for the catabolism of serine via Dglycerate stems from the existence of patients (1 l ) with L-glyceric aciduria and a partial deficiency of D-glycerate dehydrogenase (enzyme 6), although the flux through this pathway should be small, in view of the low activities of D-glycerate kinase in human as compared to rat liver (12,13). The absence of L-glyceric acid excretion in patients with D-glyceric acidemia provides an indication that their D-glycerate dehydrogenase activity is normal.…”

Section: Discussionmentioning

confidence: 99%

D-Glyceric Acidemia: An Inborn Error Associated with Fructose Metabolism

et al. 1987

View full text Add to dashboard Cite

ABSTRACT. A mentally retarded girl with epileptic sei-CASE REPORT zures is described. urinary organic acid screening ievealed a massive excretion of glyceric acid, a normally barely detectable metabolite. Hyperglycinemia was not observed. Capillary gas chromatography of the 0-acetylated (-)-menthyl ester of urinary glyceric acid showed the substance to have the D-configuration. The urinary D-glycerate excretion remained unaltered after an oral load with 200 mg/kg L-serine, but oral loading with fructose (1 g/kg) or dihydroxyacetone (1 g/kg) caused a sharp increase of the Dglycerate excretion. Treatment with a diet moderately restricted in fructose led to some clinical improvement as judged by subjective criteria. The metabolic lesion is thought to be located at some step of the fructose catabolic pathway, possibly at the level of hepatic triokinase deficiency. (Pediatr Res 21: 502-506, 1987) Abbreviations GAPD, glyceraldehyde-3-phosphate dehydrogenase NKH, nonketotic hyperglycinemia TMS, trimethylsilyl Hb, hemoglobin D-Glyceric acidemia seems to be an extremely rare inborn error of metabolism. To date only three isolated cases have been reported (1-3). The clinical presentation of the patients varied: the patient described by Wadman et al. (2) had a chronic metabolic acidosis and only a moderate developmental delay, whereas the patients described by Brandt et al. (I) and Grandgeorge et al. (3) showed all characteristic symptoms of NKH such as extreme hypotonia, deep mental retardation, myoclonic jerks, and grand ma1 seizures. Brandt's patient not only had the clinical signs of NKH, but actually had elevated levels of glycine in his plasma and urine. The concentration of D-glycerate in urine of the previously described patients ranged from 1-1 16 mmol/liter.A clear-cut enzyme defect leading to D-glyceric acidemia has not yet been found, although K$lvraa et al. (4) found evidence for a decreased activity of D-glycerate dehydrogenase in the fresh leukocytes of Brandt's patient. Enzyme studies were not performed in the other two patients.Herein we describe a patient with ~-glyceric acidemia. Results of biochemical investigations as well as of clinical studies, designed to obtain more insight in the metabolic defect, will be given.Received June 2, 1986; accepted December 19, 1986. Correspondence Dr. M. Duran, University Children's Hospital, Het Wilhelmina Kinderziekenhuis, Nieuwe Gracht 137, 3512 LK Utrecht, The Netherlands.The proband B., the first child of healthy, nonrelated parents, was born at home after an uneventful gestation of 38 wk; her birth weight was 2800 g. There were signs of perinatal asphyxia: crying started late. Apgar scores were not recorded. The mother's next pregnancy ended with a spontaneous abortion at 1 1 wk; the third pregnancy resulted in the birth of a child with spina bifida who died in the neonatal period. Subsequently two healthy children were born.In the first weeks of life the patient was noted to be hypotonic and she had severe feeding difficulties. Motor development was slo...

show abstract

Section: Discussionmentioning

confidence: 99%

D-Glyceric Acidemia: An Inborn Error Associated with Fructose Metabolism

et al. 1987

View full text Add to dashboard Cite

show abstract

“…Rat liver mitochondria-were isolated by differential centrifugation [17]. DNA was extracted by the SDS-phenol method [18] and its purity was checked by agarose electrophoresis.…”

Section: Cloning Of Rat Mitochondrial Dna Fragmentsmentioning

confidence: 99%

Enhanced transcription of mitochondrial genes after growth stimulation and glucocorticoid treatment of Reuber hepatoma H‐35

Kadowaki

Kitagawa

1988

FEBS Letters

Self Cite

View full text Add to dashboard Cite

When a serum-deprived culture of Reuber hepatoma H-35 cells was stimulated by serum, cells proliferated synchronously showing a peak of pH]thymidine incorporation within 16 h and doubling of cell number within 24 h. A 5-10-fold increase of mRNAs of mitochondrial genes was observed prior to S-phase by Northern hybridization of total cellular RNA with cloned fragments of rat mitochondrial DNA as probes. In organelle transcription by mitochondria isolated from growtharrested or growth-stimulated cells suggested that the increase of mitochondrial mRNAs was mainly due to enhanced transcription. Less enhanced transcription was observed by treating the cells with dexamethasone, which causes translocation of large amount of protein into mitochondria. The latter effect was counteracted by insulin.

show abstract

“…The enzyme showed an activity pattern similar to that of GlycDH and the only significant difference in activity was observed between young and old CR mice ( P < 0.03). The enzyme is considered to be of key importance in the ‘non‐phosphorylated’ pathway that leads to gluconeogenesis due to its irreversible ATP‐dependant action [33,34]. The enzyme is also at a point in the pathway where this pathway is connected to the fructose metabolism pathway via the conversion of glyceraldehyde to d ‐glycerate by the action of aldehyde dehydrogenase.…”

Section: Discussionmentioning

confidence: 99%

Serine utilization in mouse liver: Influence of caloric restriction and aging

Hagopian¹,

Ramsey²,

Weindruch³

2005

FEBS Letters

View full text Add to dashboard Cite

The influence of caloric restriction (CR) on the activities of hepatic serine metabolizing enzymes in young (3 months) and old (30 months) mice was studied. Serine dehydratase (SDH) activity increased markedly with age in both diet groups and in old mice was higher in the CR group. No effects of CR were observed in the young. Serine:pyruvate transaminase (SPT) and glycerate kinase activities were unaffected by age and diet. However, glycerate dehydrogenase activity was decreased in old CR mice but not in young CR. The results of this study show that long-term CR influenced serine utilization only in the pathway catalyzed by SDH. This suggests that in mouse liver this pathway is critical for serine utilization in gluconeogenesis, while the SPT pathway plays a minor role. The increase in SDH activity with long-term CR is consistent with sustained increase in gluconeogenesis.

show abstract

Identity of mitochondrial and cytosolic glycerate kinases in rat liver and regulation of their intracellular localization by dietary protein

Cited by 12 publications

References 27 publications

D-Glyceric Acidemia: An Inborn Error Associated with Fructose Metabolism

D-Glyceric Acidemia: An Inborn Error Associated with Fructose Metabolism

Enhanced transcription of mitochondrial genes after growth stimulation and glucocorticoid treatment of Reuber hepatoma H‐35

Serine utilization in mouse liver: Influence of caloric restriction and aging

Contact Info

Product

Resources

About