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The pleiotropic effects of creatine (Cr) are based mostly on the functions of the enzyme creatine kinase (CK) and its high-energy
product phosphocreatine (PCr). Multidisciplinary studies have established molecular, cellular, organ and somatic functions
of the CK/PCr system, in particular for cells and tissues with high and intermittent energy fluctuations. These studies include
tissue-specific expression and subcellular localization of CK isoforms, high-resolution molecular structures and structure–function
relationships, transgenic CK abrogation and reverse genetic approaches. Three energy-related physiological principles emerge,
namely that the CK/PCr systems functions as (a) an immediately available temporal energy buffer, (b) a spatial energy buffer
or intracellular energy transport system (the CK/PCr energy shuttle or circuit) and (c) a metabolic regulator. The CK/PCr
energy shuttle connects sites of ATP production (glycolysis and mitochondrial oxidative phosphorylation) with subcellular
sites of ATP utilization (ATPases). Thus, diffusion limitations of ADP and ATP are overcome by PCr/Cr shuttling, as most clearly
seen in polar cells such as spermatozoa, retina photoreceptor cells and sensory hair bundles of the inner ear. The CK/PCr
system relies on the close exchange of substrates and products between CK isoforms and ATP-generating or -consuming processes.
Mitochondrial CK in the mitochondrial outer compartment, for example, is tightly coupled to ATP export via adenine nucleotide
transporter or carrier (ANT) and thus ATP-synthesis and respiratory chain activity, releasing PCr into the cytosol. This coupling
also reduces formation of reactive oxygen species (ROS) and inhibits mitochondrial permeability transition, an early event
in apoptosis. Cr itself may also act as a direct and/or indirect anti-oxidant, while PCr can interact with and protect cellular
membranes. Collectively, these factors may well explain the beneficial effects of Cr supplementation. The stimulating effects
of Cr for muscle and bone growth and maintenance, and especially in neuroprotection, are now recognized and the first clinical
studies are underway. Novel socio-economically relevant applications of Cr supplementation are emerging, e.g. for senior people,
intensive care units and dialysis patients, who are notoriously Cr-depleted. Also, Cr will likely be beneficial for the healthy
development of premature infants, who after separation from the placenta depend on external Cr. Cr supplementation of pregnant
and lactating women, as well as of babies and infants are likely to be of benefit for child development. Last but not least,
Cr harbours a global ecological potential as an additive for animal feed, replacing meat- and fish meal for animal (poultry
and swine) and fish aqua farming. This may help to alleviate human starvation and at the same time prevent over-fishing of
oceans. 相似文献
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The estimation of creatine 总被引:4,自引:0,他引:4
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The taste of creatine and creatinine 总被引:1,自引:1,他引:0
Four experiments investigated the possibility of a taster-nontasterdimorphism for creatine and creatinine, and whether perceptionof these substances was related to sensitivity to phenylthiocarbamide(PTC). Threshold measurements, suprathreshold scaling of perceivedintensity, a cross-adaptation experiment and a category scalingtask produced consistent results. No relation to PTC was observed.In contrast to previous reports, no evidence of a taster-nontastereffect for creatine or creatinine was found. Creatine was anotably ineffective taste stimulus for all subjects tested. 相似文献
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The metabolic burden of creatine synthesis 总被引:1,自引:0,他引:1
Creatine synthesis is required in adult animals to replace creatine that is spontaneously converted to creatinine and excreted
in the urine. Additionally, in growing animals it is necessary to provide creatine to the expanding tissue mass. Creatine
synthesis requires three amino acids: glycine, methionine and arginine, and three enzymes: l-arginine:glycine amidinotransferase (AGAT), methionine adenosyltransferase (MAT) and guanidinoacetate methyltransferase (GAMT).
The entire glycine molecule is consumed in creatine synthesis but only the methyl and amidino groups, respectively, from methionine
and arginine. Creatinine loss averages approximately 2 g (14.6 mmol) for 70 kg males in the 20- to 39-year age group. Creatinine
loss is lower in females and in older age groups because of lower muscle mass. Approximately half of this creatine lost to
creatinine can be replaced, in omnivorous individuals, by dietary creatine. However, since dietary creatine is only provided
in animal products, principally in meat and fish, virtually all of the creatine loss in vegetarians must be replaced via endogenous
synthesis. Creatine synthesis does not appear to place a major burden on glycine metabolism in adults since this amino acid
is readily synthesized. However, creatine synthesis does account for approximately 40% of all of the labile methyl groups
provided by S-adenosylmethionine (SAM) and, as such, places an appreciable burden on the provision of such methyl groups, either from the
diet or via de novo methylneogenesis. Creatine synthesis consumes some 20–30% of arginine’s amidino groups, whether provided
in the diet or synthesized within the body. Creatine synthesis is, therefore, a quantitatively major pathway in amino acid
metabolism and imposes an appreciable burden on the metabolism of methionine and of arginine. 相似文献
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deGrauw TJ Cecil KM Byars AW Salomons GS Ball WS Jakobs C 《Molecular and cellular biochemistry》2003,244(1-2):45-48
To describe the clinical, spectroscopic and neuropsychological features of the first family diagnosed with a defect in the creatine transporter.Proton Magnetic Resonance Spectroscopy (MRS) indicated an absence of creatine and phosphocreatine in the brain of a male patient characterized by developmental delay, mild epilepsy and severe expressive language impairment. Subsequent genetic testing revealed a defect in the X-linked creatine transporter (SLC6A8/CT1), with a hemizygous mutation in the patient and a heterozygous mutation for the female carriers.Magnetic resonance imaging and spectroscopy examinations were performed on a 1.5T clinical MR Scanner. Neuropsychological examinations were performed on the index patient and maternal relatives.Preliminary spectroscopy results indicate the disorder prevents transport of creatine and phosphocreatine in the brain of the affected male. However, the skeletal muscle demonstrates the presence of creatine and phosphocreatine which correlates clinically with normal structure and function. Female carriers demonstrated impairments in confrontational naming and verbal memory assessments.This new neurological syndrome is associated with developmental delay, mild epilepsy, severe language impairment. MR Spectroscopy is a non-invasive method for obtaining a preliminary diagnosis of this disorder. Muscle creatine uptake may be normal in this disorder. 相似文献
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Muscle creatine kinase (CK) is a crucial enzyme in energy metabolism, and it exists in two forms, the reduced form (R-CK) and the oxidized form (O-CK). In contrast with R-CK, O-CK contained an intrachain disulfide bond in each subunit. Here we explored the properties of O-CK and its regulatory role on muscle CK. The intrachain disulfide bond in O-CK was demonstrated to be formed between Cys(74) and Cys(146) by site-directed mutagenesis. Biophysical analysis indicated that O-CK showed decreased catalytic activity and that it might be structurally unstable. Further assays through guanidine hydrochloride denaturation and proteolysis by trypsin and protease K revealed that the tertiary structure of O-CK was more easily disturbed than that of R-CK. Surprisingly, O-CK, unlike R-CK, cannot interact with the M-line protein myomesin through biosensor assay, indicating that O-CK might have no role in muscle contraction. Through in vitro ubiquitination assay, CK was demonstrated to be a specific substrate of muscle ring finger protein 1 (MURF-1). O-CK can be rapidly ubiquitinated by MURF-1, while R-CK can hardly be ubiquitinated, implying that CK might be degraded by the ATP-ubiquitin-proteasome pathway through the generation of O-CK. The results above were further confirmed by molecular modeling of the structure of O-CK. Therefore, it can be concluded that the generation of O-CK was a negative regulation of R-CK and that O-CK might play essential roles in the molecular turnover of MM-CK. 相似文献
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THOMAS J 《The Biochemical journal》1956,62(2):335-339
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