Plasma immunoreactive cationic trypsin(ogen) pattern in reserpinized rat model of cystic fibrosis

1996 ◽  
Vol 41 (5) ◽  
pp. 853-858 ◽  
Author(s):  
Zvi Weizman
Keyword(s):  
Cystic Fibrosis ◽  
Rat Model ◽  
Cationic Trypsin

Morphological and histochemical changes in intestinal mucosa in the reserpine-treated rat model of cystic fibrosis

1987 ◽  
Vol 47 (1) ◽  
pp. 1-12 ◽  
Author(s):  
Carol M. Park ◽  
Philip E. Reid ◽  
David A. Owen ◽  
John M. Sanker ◽  
Derek A. Applegarth
Keyword(s):  
Cystic Fibrosis ◽  
Intestinal Mucosa ◽  
Rat Model ◽  
Histochemical Changes

scholarly journals Alterations of Pancreatic Growth and of GP-2 Content in the Reserpinized Rat Model of Cystic Fibrosis

1989 ◽  
Vol 25 (5) ◽  
pp. 478-481 ◽  
Author(s):  
Francois A Leblond ◽  
Jean Morisset ◽  
Denis Lebel
Keyword(s):  
Cystic Fibrosis ◽  
Rat Model ◽  
Pancreatic Growth

Alterations of pancreatic amylase secretion in the reserpinized rat model of cystic fibrosis

1994 ◽  
Vol 16 (1) ◽  
pp. 37-44
Author(s):  
Jean Morisset ◽  
France-Line Béruhé ◽  
Micheline Vanier ◽  
Ouhida Benrezzak
Keyword(s):  
Cystic Fibrosis ◽  
Rat Model ◽  
Amylase Secretion ◽  
Pancreatic Amylase

scholarly journals Single-Dose Lentiviral Mediated Gene Therapy Recovers CFTR Function in Cystic Fibrosis Knockout Rats

2021 ◽  
Vol 12 ◽  
Author(s):  
Nicole Reyne ◽  
Patricia Cmielewski ◽  
Alexandra McCarron ◽  
Juliette Delhove ◽  
David Parsons ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Gene Therapy ◽  
Ion Transport ◽  
Rat Model ◽  
Nasal Epithelium ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Epitope Tag ◽  
Cf Transmembrane Conductance Regulator ◽  
First Time

Cystic fibrosis (CF) is a genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, resulting in defective ion transport in the airways. Addition of a functioning CFTR gene into affected airway cells has the potential to be an effective treatment for lung disease. The therapeutic efficacy of airway gene transfer can be quantified in animal models by assessing ion transport in the treated nasal epithelium using the nasal potential difference (PD) measurement technique. The nasal PD technique is routinely used in CF mice, however when applied to a recently developed CF rat model those animals did not tolerate the initial nasal PD assessment, therefore the procedure was firstly optimised in rats. This study evaluated the effect of lentiviral (LV)-mediated CFTR airway gene delivery on nasal PD in a CFTR knockout rat model. LV gene vector containing the CFTR gene tagged with a V5 epitope tag (LV-V5-CFTR) was delivered to the nasal epithelium of CF rats, and one week later nasal PD was analysed. This study demonstrated for the first time that LV-V5-CFTR treatment produced a mean correction of 46% towards wild-type chloride response in treated CF rats. Transduced cells were subsequently identifiable using V5 immunohistochemical staining. These findings in the nose validate the use of airway gene therapy for future lung based experiments.

scholarly journals A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay

2020 ◽  
Vol 11 ◽  
Author(s):  
Jyoti Sharma ◽  
Joseph Abbott ◽  
Lauren Klaskala ◽  
Guojun Zhao ◽  
Susan E. Birket ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Rat Model ◽  
Tooth Enamel ◽  
Short Circuit ◽  
Functional Restoration ◽  
Mrna Levels ◽  
Nonsense Mutations ◽  
Nonsense Mediated Decay ◽  
Rat Models

Nonsense mutations that lead to the insertion of a premature termination codon (PTC) in the cystic fibrosis transmembrane conductance regulator (CFTR) transcript affect 11% of patients with cystic fibrosis (CF) worldwide and are associated with severe disease phenotype. While CF rat models have contributed significantly to our understanding of CF disease pathogenesis, there are currently no rat models available for studying CF nonsense mutations. Here we created and characterized the first homozygous CF rat model that bears the CFTR G542X nonsense mutation in the endogenous locus using CRISPR/Cas9 gene editing. In addition to displaying severe CF manifestations and developmental defects such as reduced growth, abnormal tooth enamel, and intestinal obstruction, CFTR G542X knockin rats demonstrated an absence of CFTR function in tracheal and intestinal sections as assessed by nasal potential difference and transepithelial short-circuit current measurements. Reduced CFTR mRNA levels in the model further suggested sensitivity to nonsense-mediated decay, a pathway elicited by the presence of PTCs that degrades the PTC-bearing transcripts and thus further diminishes the level of CFTR protein. Although functional restoration of CFTR was observed in G542X rat tracheal epithelial cells in response to single readthrough agent therapy, therapeutic efficacy was not observed in G542X knockin rats in vivo. The G542X rat model provides an invaluable tool for the identification and in vivo validation of potential therapies for CFTR nonsense mutations.

scholarly journals 7: Reduced trabecular bone growth in an adolescent female cystic fibrosis rat model

2021 ◽  
Vol 20 ◽  
pp. S4
Author(s):  
M. Awadalla ◽  
E. Perilli ◽  
S. Martelli ◽  
K. Morgan ◽  
M. Kitchen ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Trabecular Bone ◽  
Rat Model ◽  
Bone Growth ◽  
Adolescent Female

Pregnancy in cystic fibrosis of the pancreas

JAMA ◽  
1966 ◽  
Vol 195 (12) ◽  
pp. 993-1000 ◽  
Author(s):  
R. J. Grand
Keyword(s):  
Cystic Fibrosis

Freeze-Fracture Examination of Tight Junctions of Human Eccrine Sweat Glands

1980 ◽  
Vol 38 ◽  
pp. 634-635
Author(s):  
J. V. Briggman ◽  
J. Bigelow ◽  
H. Bank ◽  
S. S. Spicer
Keyword(s):  
Cystic Fibrosis ◽  
Freeze Fracture ◽  
Sweat Glands ◽  
Hypertonic Solution ◽  
Dark Cells ◽  
Clear Cells ◽  
Junctional Complexes ◽  
Secretory Coil ◽  
Eccrine Sweat Glands ◽  
Eccrine Sweat

The prevalence of strands shown by freeze-fracture in the zonula occludens of junctional complexes is thought to correspond closely with the transepi-thelial electrical resistance and with the tightness of the junction and its obstruction to paracellular flow.1 The complexity of the network of junc¬tional complex strands does not appear invariably related to the degree of tightness of the junction, however, as rabbit ileal junctions have a complex network of strands and are permeable to lanthanum. In human eccrine sweat glands the extent of paracellular relative to transcellular flow remains unknown, both for secretion of the isotonic precursor fluid by the coil and for resorption of a hypertonic solution by the duct. The studies reported here undertook, therefore, to determine with the freeze-fracture technique the complexity of the network of ridges in the junctional complexes between cells in the secretory coil and the sweat ducts. Glands from a patient with cystic fibrosis were also examined because an alteration in junctional strands could underlie the decreased Na+ resorption by sweat ducts in this disease. Freeze-fracture replicas were prepared by standard procedures on isolated coil and duct segments of human sweat glands. Junctional complexes between clear cells, between dark cells and between clear and dark cells on the main lumen, and between clear cells on intercellular canaliculi of the coil con¬tained abundant anastomosing closely spaced strands averaging 6.4 + 0.7 (mean + SE) and 9.0 +0.5 (Fig. 1) per complex, respectively. Thus, the junctions in the intercellular canaliculi of the coil appeared comparable in complexity to those of tight epithlia. Occasional junctions exhibited, in addition, 2 to 5 widely spaced anastomosing strands in a very close network basal to the compact network. The fewer junctional complexes observed thus far between the superficial duct cells consisted on the average of 6 strands arranged in a close network and 1 to 4 underlying strands that lay widely separated from one another (Fig. 2). The duct epitelium would, thus, be judged slightly more "leaky" than the coil. Infrequent junctional complexes observed to date in the secretory coil segment of a cystic fibrosis specimen disclosed rela¬tively few closely crowded strands.

Chronic airway colonization by Penicillium emersonii in a patient with cystic fibrosis

1999 ◽  
Vol 37 (4) ◽  
pp. 291-293 ◽  
Author(s):  
B. Cimon ◽  
J. Carrere ◽  
J. P. Chazalette ◽  
J. F. Vinatier ◽  
D. Chabasse ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Airway Colonization ◽  
Chronic Airway
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