Estimation of the Hemoglobin Glycation Rate Constant

Kameyama, Masashi; Okumiya, Toshika; Tokuhiro, Shinji; Matsumura, Yuichi; Matsui, Hirotaka; Ono, Yasuhiro; Iwasaka, Tsuyoshi; Hiratani, Kazuyuki; Koga, Masafumi

doi:10.1101/652818

Cited by 2 publications

(2 citation statements)

References 17 publications

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“…5A ). 36 The low mean HbA1c level of the patients indicated approximately halved erythrocyte lifespans. There were no alterations in bone marrow-derived leukocytes ( Fig.…”

Section: Resultsmentioning

confidence: 93%

The motor system is exceptionally vulnerable to absence of the ubiquitously expressed superoxide dismutase-1

Park

Nordström

Tsiakas

et al. 2022

Brain Communications

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Superoxide dismutase-1 is a ubiquitously expressed antioxidant enzyme. Mutations in SOD1 can cause amyotrophic lateral sclerosis, probably via a toxic gain of function involving protein aggregation and prion-like mechanisms. Recently, homozygosity for loss-of-function mutations in SOD1 has been reported in patients presenting with infantile onset motor neuron disease. We explored the bodily effects of Superoxide dismutase-1 enzymatic deficiency in eight children homozygous for the p.C112Wfs*11 truncating mutation. In addition to physical and imaging examinations, we collected blood, urine and skin fibroblast samples. We used a comprehensive panel of clinically established analyses to assess organ function and analysed oxidative stress markers, antioxidant compounds, and the characteristics of the mutant Superoxide dismutase-1. From around 8 months of age, all patients exhibited progressive signs of both upper and lower motor neuron dysfunction, cerebellar, brain stem, and frontal lobe atrophy and elevated plasma neurofilament concentration indicating ongoing axonal damage. The disease progression seemed to slow down over the following years. The p.C112Wfs*11 gene product is unstable, rapidly degraded and no aggregates were found in fibroblast. Most laboratory tests indicated normal organ integrity and only a few modest deviations were found. The patients displayed anaemia with shortened survival of erythrocytes containing decreased levels of reduced glutathione. A variety of other antioxidants and oxidant damage markers were within normal range. In conclusion, nonneuronal organs in humans show a remarkable tolerance to absence of Superoxide dismutase-1 enzymatic activity. The study highlights the enigmatic specific vulnerability of the motor system to both gain-of-function mutations in SOD1 and loss of the enzyme as in the here depicted Infantile Superoxide dismutase-1 Deficiency Syndrome.

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“…5A ). 36 The low mean HbA1c level of the patients indicated approximately halved erythrocyte lifespans. There were no alterations in bone marrow-derived leukocytes ( Fig.…”

Section: Resultsmentioning

confidence: 93%

The motor system is exceptionally vulnerable to absence of the ubiquitously expressed superoxide dismutase-1

Park

Nordström

Tsiakas

et al. 2022

Brain Communications

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“…The current study successfully established a reliable method of estimating MRBC from EC based on a creatine model (Supplement). We would be able to determine a glycation constant for the method to estimate erythrocyte mean age from HbA1c and average glucose [14].…”

Section: Discussionmentioning

confidence: 99%

A novel method for calculating mean erythrocyte age using erythrocyte creatine

Kameyama

Koga²,

Okumiya

2020

Aging

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Estimating the lifespan of erythrocytes is useful for the differential diagnosis of anemia. However, measuring the lifespan of erythrocytes was very difficult; therefore, it was seldom measured. Erythrocyte creatine (EC) decreases reflecting erythrocyte age. We developed a method to obtain mean erythrocyte age (MRBC) from EC. We reanalyzed the previously published data from 21 patients with hemolytic anemia, which included EC and the half-life of 51 Cr. MRBC and loge EC showed excellent significant linearity (r = −0.9475, p < 0.001), proving that it could be treated as a mono-exponential relationship within the studied range (EC: 1.45-11.76 µmol/g Hb). We established an equation to obtain MRBC (days) from EC (µmol/g Hb): MRBC = −22.84loge EC + 65.83. This equation allowed calculation of MRBC based on EC which has practical applications such as the diagnosis of anemia.

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Estimation of the Hemoglobin Glycation Rate Constant

Cited by 2 publications

References 17 publications

The motor system is exceptionally vulnerable to absence of the ubiquitously expressed superoxide dismutase-1

The motor system is exceptionally vulnerable to absence of the ubiquitously expressed superoxide dismutase-1

A novel method for calculating mean erythrocyte age using erythrocyte creatine

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