Expression of the Caeruloplasmin Gene in the Adult and Neonatal Rat Liver

1989 ◽  
Vol 77 (3) ◽  
pp. 259-263 ◽  
Author(s):  
L. Barrow ◽  
M. S. Tanner ◽  
D. R. Critchley
Keyword(s):  
Steady State ◽  
Rat Liver ◽  
Messenger Rna ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Serum Copper ◽  
Neonatal Rat ◽  
Neonatal Rats ◽  
Significant Difference ◽  
Steady State Levels

1. It has been suggested that low levels of serum caeruloplasmin in Wilson's disease result from the failure to switch from a fetal to an adult mode of caeruloplasmin gene expression. To investigate postnatal expression of the caeruloplasmin gene, steady-state levels of caeruloplasmin messenger RNA in adult and neonatal rat liver were measured. 2. Copper parameters observed in neonatal rats were similar to those seen in Wilson's disease: hepatic copper concentration was significantly elevated (neonatal 164 ± 35 μg/g, adults 50 ± 8 μg/g, P < .001) and serum copper and caeruloplasmin levels were low (neonatal 0.5 ± 0.1 μg/ml, adults 1.3 ± 0.2 μg/ml, P < .001; neonatal 0.20 ± 0.04 arbitrary units, adults 0.69 ± 0.16 arbitrary units, P < .001), respectively. 3. Caeruloplasmin messenger RNA levels were analysed by Northern and dot blotting using a 12P-labelled caeruloplasmin complementary DNA probe. A caeruloplasmin messenger RNA of approximately 4.4 kilobases was detected in both adult and neonatal rat liver, with no significant difference observed in steady-state levels. 4. A step subsequent to caeruloplasmin gene transcription must therefore be impaired in neonatal rats.

scholarly journals Synaptic Injury in the Thalamus Accompanies White Matter Injury in Hypoxia/Ischemia-Mediated Brain Injury in Neonatal Rats

2019 ◽  
Vol 2019 ◽  
pp. 1-10
Author(s):  
Na Liu ◽  
Xin Tong ◽  
Wanjie Huang ◽  
Jianhua Fu ◽  
Xindong Xue
Keyword(s):  
White Matter ◽  
White Matter Injury ◽  
Neonatal Rat ◽  
Morris Water Maze Test ◽  
Neonatal Rats ◽  
Sprague Dawley ◽  
Artery Ligation ◽  
Compensatory Response ◽  
Significant Difference ◽  
Hypoxia Ischemia

The broad spectrum of disabilities caused by white matter injury (WMI) cannot be explained simply by hypomyelination. Synaptic injury in the thalamus may be related to disabilities in WMI survivors. Neuronal injury in the thalamus has been found most commonly in autopsy cases of preterm WMI. We hypothesized that hypoxia/ischemia (HI) in neonatal rats results in synaptic abnormalities in the thalamus that contribute to disabilities in WMI survivors. We examined changes in synapses in a neonatal rat model of HI-induced WMI. Right common carotid artery ligation and hypoxia (8% oxygen for 2.5 hours (h)) were performed in three-day-old Sprague-Dawley rats. We found HI rats performed worse in the Morris water maze test than sham rats, suggesting long-term cognition impairment after HI injury. A loss of synapses in the thalamus accompanied by hypomyelination and oligodendrocytes (OLs) reduction was observed. At the ultrastructural level, reductions in active zone (AZ) length and postsynaptic density (PSD) thickness were detected at 2 weeks after HI exposure. Furthermore, increased expression of synaptophysin and PSD-95 in both groups was observed from 3 days (d) to 21 d after hypoxic/ischemic (HI) injury. PSD-95 expression was significantly lower in HI rats than in sham rats from 14 d to 21 d after HI injury, and synaptophysin expression was significantly lower in HI rats from 7 d to 14 d after HI injury. However, no significant difference in synaptophysin expression was observed between HI rats and sham rats at 21 d after HI injury. The results demonstrated synaptic abnormalities in the thalamus accompanied by hypomyelination in WMI in response to HI exposure, which may contribute to the diverse neurological defects observed in WMI patients. Although synaptic reorganization occurred as a compensatory response to HI injury, the impairments in synaptic transmission were not reversed.

scholarly journals Wilson's disease: the importance of measuring serum caeruloplasmin non-immunologically

2003 ◽  
Vol 40 (2) ◽  
pp. 115-121 ◽  
Author(s):  
J. M. Walshe
Keyword(s):  
Wilson’S Disease ◽  
Liver Function Tests ◽  
Wilson's Disease ◽  
Serum Copper ◽  
Full Blood Count ◽  
Total Serum ◽  
Clotting Factors ◽  
Urinary Copper ◽  
Specialist Centre ◽  
Close Relatives

Wilson's disease should be considered as a possible diagnosis in any child, adolescent or young adult with liver damage without other explanation, especially when haemolysis is present. However, it may also present in adolescents or young adults with neurological signs confined to the motor system. The first diagnostic screening test is the estimation of the serum caeruloplasmin and total serum copper concentrations, with calculation of the serum non-caeruloplasmin-bound ('free') copper. Serum caeruloplasmin, which contains copper, is best determined by measurement of its oxidase activity, as the immunonephelometric method measures both caeruloplasmin and the biologically inactive apo-form. Diagnosis may be confirmed by an elevated urinary copper excretion. All close relatives of an identified patient must be screened and, where doubt persists, investigation of the Wilson's gene at chromosome 13q14.3 can be employed. Lifelong follow-up studies are best conducted in a specialist centre. Compliance with chelating therapy (penicillamine or trientine) or administration of the metal antagonist tetrathiomolybdate or zinc is monitored by determination of the serum 'free' copper, which should be maintained at or near 1·6 µmol/L (10 µg/100 mL). Side-effects of therapy are detected by the estimation of urinary total protein, full blood count and erythrocyte sedimentation rate, clotting factors and liver function tests.

scholarly journals Regulation of Pituitary Corticotropin-Releasing Hormone-Binding Protein Messenger Ribonucleic Acid Levels by Restraint Stress and Adrenalectomy**This work is supported by National Institutes of Health Grant DK-42730 (to A.F.S) and by a Young Investigator Award (to A.F.S.) from the National Alliance for Research on Schizophrenia and Depression.

Endocrinology ◽  
1998 ◽  
Vol 139 (11) ◽  
pp. 4435-4441 ◽  
Author(s):  
Shanna J. McClennen ◽  
Daniel N. Cortright ◽  
Audrey F. Seasholtz
Keyword(s):  
Steady State ◽  
Stress Response ◽  
Messenger Rna ◽  
Restraint Stress ◽  
Adrenal Axis ◽  
Acute Restraint Stress ◽  
Steady State Levels ◽  
Hypothalamus Pituitary Adrenal Axis ◽  
Pituitary Adrenal Axis ◽  
Acth Release

Abstract CRH is the primary hypothalamic regulator of the stress response in higher organisms, where it acts as the key mediator of ACTH release in the hypothalamus-pituitary-adrenal axis. The 37-kDa CRH-binding protein (CRH-BP) is known to bind CRH and antagonize CRH-induced ACTH release in vitro. The expression of this protein in anterior pituitary corticotrophs suggests a role for CRH-BP in modulation of the stress response. To investigate the in vivo role of rat CRH-BP, the regulation of pituitary CRH-BP gene expression by acute restraint stress and/or adrenalectomy was examined using ribonuclease protection assays. After restraint stress, steady-state levels of CRH-BP transcripts increase two to three times over basal level and remain significantly higher than basal levels for 120 min after the start of restraint. Adrenalectomy decreases CRH-BP messenger RNA steady-state levels to 8% of control levels. These results demonstrate that pituitary CRH-BP messenger RNA levels are increased in response to acute restraint stress and that glucocorticoids play a significant role in this positive regulation. These data also suggest that increased CRH-BP levels, in response to stress, may modulate the endocrine stress response by providing an additional feedback mechanism to maintain homeostasis of the hypothalamus-pituitary-adrenal axis.

scholarly journals Investigation of corneal topographic and densitometric properties of Wilson's disease patients with or without a Kayser-Fleischer ring

2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Mehmet Fuat Alakus ◽  
Mehtap Caglayan ◽  
Nazım Ekin ◽  
Hasan Oncul ◽  
Esref Arac ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Imaging System ◽  
Corneal Thickness ◽  
Wilson's Disease ◽  
Corneal Transparency ◽  
Group Iii ◽  
Corneal Densitometry ◽  
Group I ◽  
Significant Difference ◽  
Group Ii

Abstract Background To investigate the topographic measurements and densitometry of corneas in Wilson’s disease (WD) patients with or without a Kayser-Fleischer ring (KF-r) compared to healthy individuals. Methods This cross-sectional study included 20 WD patients without a KF-r (group I), 18 WD patients with a KF-r (group II), and 20 age-matched controls (group III). The Pentacam high resolution imaging system is used to determine corneal topographic measurements and densitometry. Results Mean age for groups I, II and III was 25.40 ± 6.43 years (14–36 years), 25.38 ± 6.96 years (16–39 years), 23.60 ± 6.56 years (17–35 years), respectively (P = 0.623). There was no significant difference between the groups in terms of the anterior corneal densitometry values (P > 0.05), while the 6–10 mm and 10–12 mm mid stroma and the 2–6 mm, 6–10 mm, and 10–12 mm posterior corneal densitometry values in group II were significantly higher than those in groups I and III (for all values, P <  0.05). However, the 10–12 mm posterior corneal densitometry values in group I were also significantly higher than those in group III (P = 0.038). The central corneal thickness (CCT), thinnest corneal thickness (tCT), and corneal volume (CV) values in groups I and II were significantly lower than those in group III (for CCT values, P = 0.011 and P = 0.009; for tCT values, P = 0.010 and P = 0.005; for CV values, P = 0.043 and P = 0.029). Conclusion In WD patients with a KF-r, corneal transparency decreased in the peripheral posterior and mid stromal corneal layers; for these patients, corneal transparency may be impaired not only in the peripheral cornea but also in the paracentral cornea.

scholarly journals Wilson’s Disease Presenting in Late Adult Life

2021 ◽  
pp. 142-146
Author(s):  
Wafa AlDhaleei ◽  
Maryam AlAhmad ◽  
Ibrahim Alhosani
Keyword(s):  
Wilson’S Disease ◽  
Adult Life ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Serum Copper ◽  
Hepatic Copper ◽  
Urinary Copper ◽  
Clinical Presentations ◽  
Function Impairment ◽  
Diagnostic Management

Wilson’s disease (WD) is an autosomal recessive disease affecting the copper metabolism resulting in various clinical presentations. Diagnosis includes the presence of low serum copper and ceruloplasmin concentrations, increased urinary copper excretion, and/or increased hepatic copper concentrations. Yet, genetic testing remains diagnostic. Management includes copper chelating agents and liver transplant in advance cases. We report a case of WD presenting with liver function impairment in late adult life and started on treatment. Therefore, early diagnosis and treatment of WD can prevent related complications.

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