Atypical Presentation of the GLUT-1 Deficiency Syndrome with Cataract and Normal Glucose Concentration in Cerebrospinal Fluid

2014 ◽  
Vol 45 (S 01) ◽  
Author(s):  
N. Schmitz ◽  
A. Blank ◽  
M. Bartels ◽  
R. König ◽  
M. Kieslich
Keyword(s):  
Cerebrospinal Fluid ◽  
Glucose Concentration ◽  
Deficiency Syndrome ◽  
Atypical Presentation ◽  
Glut 1 ◽  
Normal Glucose

scholarly journals High glucose induces early senescence in adipose-derived stem cells by accelerating p16 and mTOR

2019 ◽  
Vol 6 (6) ◽  
pp. 3213-3221
Author(s):  
Hieu Liem Pham ◽  
Phuc Van Pham
Keyword(s):  
Stem Cells ◽  
High Glucose ◽  
Glucose Concentration ◽  
Culture Medium ◽  
Diabetic Patients ◽  
Adipose Derived Stem Cells ◽  
Differentiation Potential ◽  
Normal Glucose

Introduction: The senescence of stem cells is the primary reason that causes aging of stem cell-containing tissues. Some hypotheses have suggested that high glucose concentration in diabetic patients is the main factor that causes senescence of cells in those patients. This study aimed to evaluate the effects of high glucose concentrations on the senescence of adipose-derived stem cells (ADSCs). Methods: ADSCs were isolated and expanded from human adipose tissues. They were characterized and confirmed as mesenchymal stem cells (MSCs) by expression of surface markers, their shape, and in vitro differentiation potential. They were then cultured in 3 different media- that contained 17.5 mM, 35 mM, or 55 mM of D-glucose. The senescent status of ADSCs was recorded by the expression of the enzyme beta-galactosidase, cell proliferation, and doubling time. Real-time RT-PCR was used to evaluate the expression of p16, p21, p53 and mTOR. Results: The results showed that high glucose concentrations (35 mM and 55 mM) in the culture medium induced senescence of human ADSCs. The ADSCs could progress to the senescent status quicker than those cultured in the lower glucose-containing medium (17.5 mM). The senescent state was related to the up-regulation of p16 and mTOR genes. Conclusion: These results suggest that high glucose in culture medium can trigger the expression of p16 and mTOR genes which cause early senescence in ADSCs. Therefore, ADSCs should be cultured in low glucose culture medium, or normal glucose concentration, to extend their life in vitro as well as in vivo.  

scholarly journals A Different SLC2A1 Gene Mutation in Glut 1 Deficiency Syndrome: c.734A>C

2017 ◽  
Vol 34 (6) ◽  
pp. 580-583 ◽  
Author(s):  
Rüya Çolak ◽  
Senem Alkan Özdemir ◽  
Ezgi Yangın Ergon ◽  
Mehtap Kağnıcı ◽  
Şebnem Çalkavur
Keyword(s):  
Gene Mutation ◽  
Deficiency Syndrome ◽  
Glut 1 ◽  
Slc2a1 Gene

Muscle glucose transport and phosphorylation in type 2 diabetic, obese nondiabetic, and genetically predisposed individuals

2007 ◽  
Vol 292 (1) ◽  
pp. E92-E100 ◽  
Author(s):  
Merri Pendergrass ◽  
Alessandra Bertoldo ◽  
Riccardo Bonadonna ◽  
Gianluca Nucci ◽  
Lawrence Mandarino ◽  
...  
Keyword(s):  
Glucose Transport ◽  
Brachial Artery ◽  
Glucose Concentration ◽  
Whole Body ◽  
Insulin Resistant ◽  
Normal Glucose ◽  
Glucose Phosphorylation ◽  
Forearm Muscle ◽  
Type 2 Diabetic

Our objectives were to quantitate insulin-stimulated inward glucose transport and glucose phosphorylation in forearm muscle in lean and obese nondiabetic subjects, in lean and obese type 2 diabetic (T2DM) subjects, and in normal glucose-tolerant, insulin-resistant offspring of two T2DM parents. Subjects received a euglycemic insulin (40 mU·m−2·min−1) clamp with brachial artery/deep forearm vein catheterization. After 120 min of hyperinsulinemia, a bolus of d-mannitol/3- O-methyl-d-[14C]glucose/d-[3-3H]glucose (triple-tracer technique) was given into brachial artery and deep vein samples obtained every 12–30 s for 15 min. Insulin-stimulated forearm glucose uptake (FGU) and whole body glucose metabolism (M) were reduced by 40–50% in obese nondiabetic, lean T2DM, and obese T2DM subjects (all P < 0.01); in offspring, the reduction in FGU and M was ∼30% ( P < 0.05). Inward glucose transport and glucose phosphorylation were decreased by ∼40–50% ( P < 0.01) in obese nondiabetic and T2DM groups and closely paralleled the decrease in FGU. The intracellular glucose concentration in the space accessible to glucose was significantly greater in obese nondiabetic, lean T2DM, obese T2DM, and offspring compared with lean controls. We conclude that 1) obese nondiabetic, lean T2DM, and offspring manifest moderate-to-severe muscle insulin resistance (FGU and M) and decreased insulin-stimulated glucose transport and glucose phosphorylation in forearm muscle; these defects in insulin action are not further reduced by the combination of obesity plus T2DM; and 2) the increase in intracelullar glucose concentration under hyperinsulinemic euglycemic conditions in obese and T2DM groups suggests that the defect in glucose phosphorylation exceeds the defect in glucose transport.

scholarly journals Nonconvulsive Status Epilepticus on Electroencephalography: An Atypical Presentation of Subacute Sclerosing Panencephalitis in Two Children

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Pratibha Singhi ◽  
Arushi Gahlot Saini ◽  
Jitendra Kumar Sahu
Keyword(s):  
Cerebrospinal Fluid ◽  
Status Epilepticus ◽  
Cognitive Decline ◽  
Neurodegenerative Disease ◽  
Slow Wave ◽  
Subacute Sclerosing Panencephalitis ◽  
Nonconvulsive Status Epilepticus ◽  
Atypical Presentation ◽  
Measles Antibodies

Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography. We report two pediatric cases who presented with periodic myoclonic jerks and cognitive decline. In both cases, the electroencephalogram showed continuous nonconvulsive status epilepticus activity. Both had elevated measles antibodies in cerebrospinal fluid and blood. Pediatricians need to be aware of this atypical presentation of subacute sclerosing panencephalitis.

GLUT-1 deficiency syndrome caused by haploinsufficiency of the blood-brain barrier hexose carrier

1998 ◽  
Vol 18 (2) ◽  
pp. 188-191 ◽  
Author(s):  
Glen Seidner ◽  
Marcela Garcia Alvarez ◽  
Jih-I Yeh ◽  
Kevin R. O'Driscoll ◽  
Jörg Klepper ◽  
...  
Keyword(s):  
Blood Brain Barrier ◽  
Deficiency Syndrome ◽  
Brain Barrier ◽  
Glut 1 ◽  
Blood Brain ◽  
Hexose Carrier

scholarly journals Regulation of angiotensin II receptors and PKC isoforms by glucose in rat mesangial cells

1999 ◽  
Vol 276 (5) ◽  
pp. F691-F699 ◽  
Author(s):  
Farhad Amiri ◽  
Raul Garcia
Keyword(s):  
Angiotensin Ii ◽  
High Glucose ◽  
Glucose Concentration ◽  
Mesangial Cells ◽  
Competitive Binding ◽  
Ang Ii ◽  
Pkc Isoforms ◽  
Normal Glucose ◽  
Elevated Glucose ◽  
In Cells

It has been shown that glomerular angiotensin II (ANG II) receptors are downregulated and protein kinase C (PKC) is activated under diabetic conditions. We, therefore, investigated ANG II receptor and PKC isoform regulation in glomerular mesangial cells (MCs) under normal and elevated glucose concentrations. MCs were isolated from collagenase-treated rat glomeruli and cultured in medium containing normal or high glucose concentrations (5.5 and 25.0 mM, respectively). Competitive binding experiments were performed using the ANG II antagonists losartan and PD-123319, and PKC analysis was conducted by Western blotting. Competitive binding studies showed that the AT1 receptor was the only ANG II receptor detected on MCs grown to either subconfluence or confluence under either glucose concentration. AT1 receptor density was significantly downregulated in cells grown to confluence in high-glucose medium. Furthermore, elevated glucose concentration enhanced the presence of all MC PKC isoforms. In addition, PKCβ, PKCγ and PKCε were translocated only in cells cultured in elevated glucose concentrations following 1-min stimulation by ANG II, whereas PKCα, PKCθ, and PKCλ were translocated by ANG II only in cells grown in normal glucose. Moreover, no changes in the translocation of PKCδ, PKCι, PKCζ, and PKCμ were detected in response to ANG II stimulation under euglycemic conditions. We conclude that MCs grown in high glucose concentration show altered ANG II receptor regulation as well as PKC isoform translocation compared with cells grown in normal glucose concentration.

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