Long-Term Therapy with NTBC and Tyrosine-Restricted Diet in a Murine Model of Hereditary Tyrosinemia Type I

2002 ◽  
Vol 75 (1) ◽  
pp. 38-45 ◽  
Author(s):  
M. Al-Dhalimy ◽  
K. Overturf ◽  
M. Finegold ◽  
M. Grompe
Keyword(s):  
Murine Model ◽  
Term Therapy ◽  
Type I ◽  
Restricted Diet ◽  
Tyrosinemia Type I ◽  
Hereditary Tyrosinemia ◽  
Long Term Therapy

scholarly journals 405. Correction of the Murine Model of Hereditary Tyrosinemia Type I Using Messenger RNA as a Source of Transposase for Sleeping Beauty Mediated Integration of the FAH Gene

2006 ◽  
Vol 13 ◽  
pp. S155-S156
Author(s):  
Andrew Wilber ◽  
Kirk J. Wangensteen ◽  
Yixin Chen ◽  
Lijuan Zhou ◽  
Joel L. Frandsen ◽  
...  
Keyword(s):  
Murine Model ◽  
Messenger Rna ◽  
Sleeping Beauty ◽  
Type I ◽  
Tyrosinemia Type I ◽  
Hereditary Tyrosinemia

scholarly journals Effect of Erythromycin on Chronic Respiratory Infection Caused by Pseudomonas aeruginosa with Biofilm Formation in an Experimental Murine Model

2004 ◽  
Vol 48 (6) ◽  
pp. 2251-2259 ◽  
Author(s):  
Towako Nagata ◽  
Hiroshi Mukae ◽  
Junichi Kadota ◽  
Tomayoshi Hayashi ◽  
Takeshi Fujii ◽  
...  
Keyword(s):  
Pseudomonas Aeruginosa ◽  
Murine Model ◽  
Biofilm Formation ◽  
Term Therapy ◽  
Diffuse Panbronchiolitis ◽  
Chronic Respiratory Infection ◽  
Model Treatment ◽  
Long Term Therapy

ABSTRACT Diffuse panbronchiolitis (DPB) is a chronic lower respiratory tract infection commonly associated with persistent late-stage Pseudomonas aeruginosa infection. However, low-dose long-term therapy with certain macrolides is effective in most patients with DPB. The present study was designed to examine the effects of long-term erythromycin (ERY) therapy by using our established murine model of chronic respiratory P. aeruginosa infection. ERY or saline was administered from day 80 after intubation with a P. aeruginosa-precoated tube for the subsequent 10, 20, 40, and 80 days. Bacteriologic and histologic analyses of the murine lungs and electron microscopy of the intubated tube were performed. In the murine model, treatment with ERY for 80 days significantly reduced the number of viable P. aeruginosa organisms in the lungs (P < 0.05). The biofilm formed in situ by P. aeruginosa on the inner wall of the inoculation tube placed into the murine bronchus became significantly thinner after 80 days of ERY treatment. We conclude that the clinical efficacy of macrolides in DPB may be due at least in part to the reduction in P. aeruginosa biofilm formation.

scholarly journals In vivo suppressor mutations correct a murine model of hereditary tyrosinemia type I

1999 ◽  
Vol 96 (21) ◽  
pp. 11928-11933 ◽  
Author(s):  
K. Manning ◽  
M. Al-Dhalimy ◽  
M. Finegold ◽  
M. Grompe
Keyword(s):  
Murine Model ◽  
Type I ◽  
Suppressor Mutations ◽  
Tyrosinemia Type I ◽  
Hereditary Tyrosinemia

scholarly journals Therapeutic Targeting of Fumaryl Acetoacetate Hydrolase in Hereditary Tyrosinemia Type I

2021 ◽  
Vol 22 (4) ◽  
pp. 1789
Author(s):  
Jon Gil-Martínez ◽  
Iratxe Macias ◽  
Luca Unione ◽  
Ganeko Bernardo-Seisdedos ◽  
Fernando Lopitz-Otsoa ◽  
...  
Keyword(s):  
Autosomal Recessive Disorder ◽  
Type I ◽  
Secondary Effects ◽  
Pharmacological Chaperones ◽  
Liver And Kidney ◽  
Tyrosinemia Type I ◽  
Fumarylacetoacetate Hydrolase ◽  
Hereditary Tyrosinemia ◽  
First Time

Fumarylacetoacetate hydrolase (FAH) is the fifth enzyme in the tyrosine catabolism pathway. A deficiency in human FAH leads to hereditary tyrosinemia type I (HT1), an autosomal recessive disorder that results in the accumulation of toxic metabolites such as succinylacetone, maleylacetoacetate, and fumarylacetoacetate in the liver and kidney, among other tissues. The disease is severe and, when untreated, it can lead to death. A low tyrosine diet combined with the herbicidal nitisinone constitutes the only available therapy, but this treatment is not devoid of secondary effects and long-term complications. In this study, we targeted FAH for the first-time to discover new chemical modulators that act as pharmacological chaperones, directly associating with this enzyme. After screening several thousand compounds and subsequent chemical redesign, we found a set of reversible inhibitors that associate with FAH close to the active site and stabilize the (active) dimeric species, as demonstrated by NMR spectroscopy. Importantly, the inhibitors are also able to partially restore the normal phenotype in a newly developed cellular model of HT1.

scholarly journals Heat Shock Response Associated with Hepatocarcinogenesis in a Murine Model of Hereditary Tyrosinemia Type I

Cancers ◽  
2014 ◽  
Vol 6 (2) ◽  
pp. 998-1019 ◽  
Author(s):  
Francesca Angileri ◽  
Geneviève Morrow ◽  
Vincent Roy ◽  
Diana Orejuela ◽  
Robert Tanguay
Keyword(s):  
Heat Shock ◽  
Murine Model ◽  
Heat Shock Response ◽  
Type I ◽  
Shock Response ◽  
Tyrosinemia Type I ◽  
Hereditary Tyrosinemia

Antithrombotic Therapy for DVT/PE

1997 ◽  
Vol 17 (03) ◽  
pp. 161-162
Author(s):  
Thomas Hyers
Keyword(s):  
Molecular Weight ◽  
Low Molecular Weight Heparin ◽  
Oral Anticoagulants ◽  
Cost Effective ◽  
Therapeutic Agents ◽  
Great Promise ◽  
Term Therapy ◽  
Low Molecular Weight ◽  
Long Term Therapy

SummaryProblems with unfractionated heparin as an antithrombotic have led to the development of new therapeutic agents. Of these, low molecular weight heparin shows great promise and has led to out-patient therapy of DVT/PE in selected patients. Oral anticoagulants remain the choice for long-term therapy. More cost-effective ways to give oral anticoagulants are needed.

Office-based post-marketing surveillance study on the influence of long term therapy with aripiprazole in patients with schizophrenia

2007 ◽  
Vol 40 (05) ◽  
Author(s):  
M Kungel ◽  
A Engelhardt ◽  
T Spevakné-Göröcs ◽  
M Ebrecht ◽  
C Werner ◽  
...  
Keyword(s):  
Surveillance Study ◽  
Term Therapy ◽  
Post Marketing Surveillance ◽  
Post Marketing ◽  
Long Term Therapy
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