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Showing 1-30 of 122 results for "M54058" within Papers
Ramon Bonte et al.
Metabolites, 9(12) (2019-11-30)
Routine diagnostic screening of inborn errors of metabolism (IEM) is currently performed by different targeted analyses of known biomarkers. This approach is time-consuming, targets a limited number of biomarkers and will not identify new biomarkers. Untargeted metabolomics generates a global
S Kölker et al.
Cellular and molecular life sciences : CMLS, 62(6), 621-624 (2005-03-17)
Methylmalonic acid was previously considered as major neurotoxin in methylmalonic acidurias. In contrast, recent studies support the notion that other metabolites deriving from propionyl-coenzyme A, inducing synergistic inhibition of mitochondrial energy metabolism, are more important than methylmalonic acid to understand
M J Bennett et al.
Annals of clinical biochemistry, 29 ( Pt 5), 541-545 (1992-09-01)
We have analysed organic acid profiles in 74 samples of post-mortem vitreous humour from the sudden infant death syndrome and compared the profiles to those obtained from the corresponding urine or bladder wall swab. There was a high degree of
S P Stabler et al.
The Journal of clinical investigation, 77(5), 1606-1612 (1986-05-01)
To determine the incidence of elevated levels of serum methylmalonic acid in patients with cobalamin deficiency, we utilized a new capillary gas chromatographic-mass spectrometric technique to measure methylmalonic acid in the serum of 73 patients with clinically confirmed cobalamin deficiency.
E Nakamura et al.
Clinica chimica acta; international journal of clinical chemistry, 68(2), 127-140 (1976-04-15)
We have developed a sensitive gas-chromatographic method for the determination of methylmalonic acid and other short chain dircarboxylic acids in biological samples. The method is based on the isolation of the short chain dicarboxylic acid fraction by Dowex 3 X
Fiona O'Leary et al.
The British journal of nutrition, 108(11), 1948-1961 (2012-10-23)
Poor vitamin B₁₂ status may lead to the development of cognitive decline and dementia but there is a large variation in the quality, design of and results reported from these investigations. We have undertaken a systematic review of the evidence
L S Kornerup et al.
Clinical nutrition (Edinburgh, Scotland), 38(2), 906-911 (2018-03-07)
Bariatric surgery increases the risk of micronutrient deficiencies, including vitamin B12 (B12) deficiency. We analysed early changes in biomarkers of B12 status following bariatric surgery. We prospectively included adult patients (n = 27) referred for either Roux-en-Y Gastric Bypass (RYGB) (n = 19) or
Tandem mass spectrometric determination of malonylcarnitine: diagnosis and neonatal screening of malonyl-CoA decarboxylase deficiency.
René Santer et al.
Clinical chemistry, 49(4), 660-662 (2003-03-26)
M A Lovell et al.
Archives of neurology, 58(3), 392-396 (2001-03-20)
Markers of oxidative stress are increased in cerebrospinal fluid (CSF) of patients with Alzheimer disease (AD), although none of those reported are appropriate diagnostic markers because of the overlap between patients with AD and control subjects. To determine the ratio
A Lindgren
Scandinavian journal of clinical and laboratory investigation, 62(1), 15-19 (2002-05-11)
Serum methylmalonic acid (S-MMA) as a sensitive indicator of cobalamin deficiency was introduced more than 10 years ago. The use of this method for identifying patients with cobalamin deficiency reflects much higher prevalence figures than was previously thought. In this
Jian-ping Jia et al.
Chinese medical journal, 117(8), 1161-1164 (2004-09-14)
An important aspect of Alzheimer's disease (AD) is loss or impairment of cholinergic neurons. It is controversial whether there is a similar cholinergic impairment and cerebral deficit of acetylcholine (ACh) in the case of vascular dementia (VD). The purpose of
[Methylmalonic acid].
A Tanaka
Nihon rinsho. Japanese journal of clinical medicine, 57 Suppl, 660-662 (1999-09-30)
S P Stabler et al.
The Journal of biological chemistry, 263(12), 5581-5588 (1988-04-25)
It has been reported (Poston, J. (1976) J. Biol. Chem. 251, 1859-1863; (1982) 255, 10067-10072; (1984) 259, 2059-2061) that mammalian tissues contain an adenosylcobalamin-dependent enzyme, leucine 2,3-aminomutase, which catalyzes the interconversion of beta-leucine and leucine. It was also reported that
D Leupold
Klinische Wochenschrift, 55(2), 57-63 (1977-01-15)
Congenital methylmalonic aciduria (MMA) is a metabolic disorder inherited by an autosomal recessive trait. The metabolic block is located in the catabolic pathway of propionyl-CoA to succinyl-CoA. Biochemically, four enzymatic defects have been recognized, i.e.: 1. Methylmalonyl-CoA racemase. 2. Methylmalonyl-CoA
[Blood and urinary level of methylmalonic acid: an important index in evaluating cobalamin deficiency].
B A Sela et al.
Harefuah, 132(12), 866-871 (1997-06-15)
S P Stabler et al.
Blood, 81(12), 3404-3413 (1993-06-15)
Homocysteine can be methylated to form methionine by the cobalamin- (Cbl) and folate-dependent enzyme, methionine synthase; serum levels of total homocysteine are elevated in greater than 95% of patients with either Cbl or folate deficiency. Homocysteine can also condense with
E P Frenkel et al.
Blood, 49(1), 125-137 (1977-01-01)
A rapid sensitive spectrophotometric assay for the measurement of methylmalonic and propionic acids in urine is described. The assay is based upon the quantitation of propionic acid using acetyl coenzyme A synthetase isolated from baker's yeast. This enzyme is highly
M G Garrod et al.
European journal of clinical nutrition, 64(5), 503-509 (2010-03-11)
A common polymorphism, C776G, in the plasma B12 transport protein transcobalamin (TC), encodes for either proline or arginine at codon 259. This polymorphism may affect the affinity of TC for B12 and subsequent delivery of B12 to tissues. TC genotype
Ana P Gomes et al.
Nature, 585(7824), 283-287 (2020-08-21)
The risk of cancer and associated mortality increases substantially in humans from the age of 65 years onwards1-6. Nonetheless, our understanding of the complex relationship between age and cancer is still in its infancy2,3,7,8. For decades, this link has largely been
Maja Klaus et al.
ACS chemical biology, 14(3), 426-433 (2019-01-27)
Engineering of assembly line polyketide synthases (PKSs) to produce novel bioactive compounds has been a goal for over 20 years. The apparent modularity of PKSs has inspired many engineering attempts in which entire modules or single domains were exchanged. In
M Vrethem et al.
Multiple sclerosis (Houndmills, Basingstoke, England), 9(3), 239-245 (2003-06-20)
The aim of this study was to evaluate if multiple sclerosis (MS) is associated with vitamin B12 (cobalamin) deficiency. We measured serum vitamin B12, plasma folate, serum methylmalonic acid (MMA), plasma homocysteine (tHcy) and also cerebrospinal fluid (CSF) MMA and
J A Molina et al.
Journal of neural transmission (Vienna, Austria : 1996), 109(7-8), 1035-1044 (2002-07-12)
Thiamine is an essential cofactor for several important enzymes involved in brain oxidative metabolism, such as the alpha-ketoglutarate dehydrogenase complex (KGDHC), pyruvate-dehydrogenase complex (PDHC), and transketolase. Some investigators reported decreased thiamine-diphosphate levels and decreased activities of KGDHC, pyruvate-dehydrogenase complex and
Yu-peng Liu et al.
Zhonghua er ke za zhi = Chinese journal of pediatrics, 50(6), 410-414 (2012-08-31)
Methylmalonic aciduria is the most common disorder of organic acidurias in the mainland of China. It is also the one of treatable metabolic disorders. The clinical spectrum of the patients varies from severe neonatal-onset forms with neonatal brain injury and
M O Kristensen et al.
Acta neurologica Scandinavica, 87(6), 475-481 (1993-06-01)
The cobalamin status was evaluated in Alzheimer dementia (n = 26), other dementias (n = 24), various gerontopsychiatric disorders (n = 25), and in neuro-psychiatrically healthy controls (n = 20). Supplementing serum cobalamin we measured methylmalonic acid (MMA), a metabolite
Sopak Supakul et al.
Journal of clinical medicine, 9(8) (2020-07-28)
Sole measurement of plasma vitamin B12 is no longer enough to identify vitamin B12 (B12) deficiency. When plasma vitamin B12 is in the low-normal range, especially between 201 and 350 ng/L, B12 deficiency should be assessed by measurements of plasma
Bryce J Marquis et al.
Chromatographia, 80(12), 1723-1732 (2017-12-08)
Small molecules containing carboxylic acid functional groups are ubiquitous throughout biology, playing vital roles in biological chemistry ranging from energy metabolism to cellular signaling. This paper describes a new derivatization reagent, 4-bromo-
G F Hoffmann et al.
Journal of inherited metabolic disease, 16(4), 648-669 (1993-01-01)
Concentrations of organic acids in cerebrospinal fluid (CSF) appear to be directly dependent upon their rate of production in the brain. There is evidence that the net release of short-chain monocarboxylic acids from the brain is a major route for
Lucy C Fox et al.
Nephrology (Carlton, Vic.), 23(6), 507-517 (2018-02-09)
Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request
Takeshi Miyazawa et al.
Nature communications, 11(1), 80-80 (2020-01-05)
To harness the synthetic power of modular polyketide synthases (PKSs), many aspects of their biochemistry must be elucidated. A robust platform to study these megadalton assembly lines has not yet been described. Here, we in vitro reconstitute the venemycin PKS
Valerio Leoni et al.
Clinical chemistry and laboratory medicine, 42(2), 186-191 (2004-04-06)
24S-Hydroxycholesterol (24OHC) and 27-hydroxycholesterol (27OHC) are two structurally similar oxysterols of different origins--the former almost exclusively formed in the brain and the latter formed to a lesser extent in the brain than in most other organs. HYPOTHESIS TO BE TESTED:
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