GABA and Taurine What Are Metabolites Like This Doing in Places Like That?

Rozen, Rima; Goodyer, Paul; Scriver, Charles R.

doi:10.1007/978-1-4684-4187-1_6

Cited by 9 publications

(5 citation statements)

References 234 publications

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“…Gamma-aminobutyric acid (GABA) is an important, main-Iy inhibitory, neurotransmitter estimated to be present in nearly one-third of human synapses (Rozen et al 1982). Two enzymatic defects in the metabolism ofGABA have been reported: one in its synthesis (a glutamic acid decarboxylase defeet) causing pyridoxine-dependent convulsions (Yoshida et al 1971), and one in its degradation (succinic semialdehyde dehydrogenase deficiency), characterized by mental retardation, hypotonia and ataxia (Jakobs et al 1981, Rating et al 1982.…”

Section: Introductlonmentioning

confidence: 99%

Gamma-Aminobutyric Acid-Transaminase Deficiency: A Newly Recognized Inborn Error of Neurotransmitter Metabolism

et al. 1984

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Cerebrospinal fluid aminoacid analysis in a girl with severe psychomotor retardation, hypotonia, hyperreflexia and growth acceleration showed highly increased levels of free gamma-aminobutyric acid (4.8 mumol/l; range in twenty controls 0.04-0.12, median 0.08), homocarnosine, a dipeptide of gamma-aminobutyric acid and histidine (23.4 mumol/l; control range 4.0-8.7, median 7.6) and of beta-alanine, an alternative substrate for gamma-aminobutyric acid-transaminase (0.48 mumol/l; control range 0.02-0.06, median 0.05). Liver gamma-aminobutyric acid-transaminase activity was deficient (0.07 mumol/mg protein h; range in ten controls 0.31-0.69, median 0.38). Fasting plasma growth hormone levels were increased (7.9-38.4 ng/ml; nl less than 5). Brain evoked responses were suggestive of leukodystrophy. A brother of this patient, showing a similar clinical picture, had died at one year. Postmortem examination of his brain showed leukodystrophy of the type seen in amino acidopathies such as phenylketonuria. This appears to be the first report of gamma-aminobutyric acid-transaminase deficiency.

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Section: Introductlonmentioning

confidence: 99%

Gamma-Aminobutyric Acid-Transaminase Deficiency: A Newly Recognized Inborn Error of Neurotransmitter Metabolism

et al. 1984

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“…In addition to its well-defined role in bile acid conjugation, it has been implicated as a membrane stabilizer and a modulator of ion fluxes in excitatory tissues (1,2).…”

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confidence: 99%

Renal transport of taurine adapts to perturbed taurine homeostasis.

Rozen¹,

Scriver²

1982

Proc. Natl. Acad. Sci. U.S.A.

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Renal adaptation apparently contributes to the homeostasis of taurine, a a-amino compound that behaves as a conserved metabolite in the mammal. We studied two strains of inbred mice: C3H/Hej (low-taurine excreter) and C57BL/6J (high-taurine excreter due to impaired basolateral membrane permeability to taurine). Low-protein and low-sulfur amino acid diets fed for two weeks significantly decreased plasma taurine in both strains, decreased fractional taurine excretion in vivo (particularly in the C57BL strain), and increased net uptake of taurine by renal cortex slices and isolated brush-border membrane vesicles (BBMV) in vitro in both strains. Renal adaptation was less obvious in vivo in the low-taurine excreter C3H strain, but in vitro adaptation, as observed in slices and BBMV (P < 0.01), was greater than that observed in the C57BL strain. Renal cellular taurine content fell (P < 0.01) only in the adapted C3H strain. The in vitro adaptive response was not confined to taurine; BBMV uptake of D-glucose and L-alanine was also enhanced in the adapted state. Specificity of the stimulus for adaptation was tested with a low-phenylalanine diet; a modest adaptation was observed in vivo and in vitro but only in the C3H strain. BBMV adaptation did not correlate with blood methionine but correlated inversely with plasma taurine (r = 0.71, P < 0.05), implying that change in extracellular taurine may be a signal for renal adaptation in taurine homeostasis in the mammal.

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“…Methionine, cysteine and cystine have not been studied as extensively as tau rine, because of the essential role of the latter in the development and normal maturation of the nervous system [7,12]. There is con siderable information about the passage of taurine through the milk during lactation [12], but the possible contribution of mater nal taurine to fetal development is more dis puted due to controversial results on the pas sage of taurine through the placenta [13][14][15].…”

Section: Introductionmentioning

confidence: 99%

“…Several metabolic functions are attrib uted to taurine: it is considered as a neurotransmitter [8], a modulator of ionic fluxes in the retina [9]; it is also believed to increase membrane permeability to ions in cardiac muscle [10], but the most specific action of taurine is to conjugate with cholesterol to form bile acids [11]. The unknown metabolization of taurine and its presence in all tis sues [1,7] support its potential essential role in metabolism.…”

Section: Introductionmentioning

confidence: 99%

“…Cysteine as an essential constituent of glutathione [1,2] and due to its -SH radical can play an essential part in the stabilization of protein structure. Accumulation of cys teine as cystine can be considered as another potential mechanism in the accumulation of -SH radicals in a nonmetabolizing form, al though high intracellular cysteine/cystine ra tios have been reported [3], Taurine is the most controversial sulphur amino acid; thus, its plasma or tissue concentrations are higher than those of other sulphur amino acids [4][5][6][7]. Several metabolic functions are attrib uted to taurine: it is considered as a neurotransmitter [8], a modulator of ionic fluxes in the retina [9]; it is also believed to increase membrane permeability to ions in cardiac muscle [10], but the most specific action of taurine is to conjugate with cholesterol to form bile acids [11].…”

Section: Introductionmentioning

confidence: 99%

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Sulphur Amino Acid Levels in Some Tissues of the Rat during Pregnancy and Lactation

López-Tejero¹,

Pastor‐Anglada²,

Remesar³

1987

Ann Nutr Metab

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Variations in free sulphur amino acid content in some rat tissues have been measured during pregnancy and lactation. As a general trend, sulphur amino acids tend to accumulate in tissues in late pregnancy. This accumulation is quantitatively more important in the liver and striated muscle than in the skin and the kidneys. Among sulphur amino acids, taurine shows the most marked changes, with a maximum accumulation in the liver and striated muscle on day 19 of pregnancy. After delivery, the levels of amino acids in the tissue pools show a tendency to drop, especially in liver and striated muscle. As expected, taurine shows the most marked decrease, returning to normal values in muscle but decreasing to depletion in the liver on day 20 after delivery. These results can be interpreted as follows: during pregnancy, increased food intake is sufficient to accommodate the metabolic needs of the mother, but during lactation, the requirements of milk synthesis promote the release of these amino acids from the tissues.

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GABA and Taurine What Are Metabolites Like This Doing in Places Like That?

Cited by 9 publications

References 234 publications

Gamma-Aminobutyric Acid-Transaminase Deficiency: A Newly Recognized Inborn Error of Neurotransmitter Metabolism

Gamma-Aminobutyric Acid-Transaminase Deficiency: A Newly Recognized Inborn Error of Neurotransmitter Metabolism

Renal transport of taurine adapts to perturbed taurine homeostasis.

Sulphur Amino Acid Levels in Some Tissues of the Rat during Pregnancy and Lactation

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