Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Clinical, biochemical, and molecular aspects of Glanzmann's thrombastheniain humans and dogs
Autore:
Boudreaux, MK; Lipscomb, DL;
Indirizzi:
Auburn Univ, Coll Vet Med, Dept Pathobiol, Auburn, AL 36849 USA Auburn Univ Auburn AL USA 36849 Med, Dept Pathobiol, Auburn, AL 36849 USA
Titolo Testata:
VETERINARY PATHOLOGY
fascicolo: 3, volume: 38, anno: 2001,
pagine: 249 - 260
SICI:
0300-9858(200105)38:3<249:CBAMAO>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
GLYCOPROTEIN-IIB-IIIA; CALCIUM-BINDING DOMAIN; PLATELET MEMBRANE-GLYCOPROTEINS; AMINO-ACID SUBSTITUTION; INTEGRIN SUBUNIT ASSOCIATION; GREAT PYRENEES DOG; LIGAND-BINDING; GPIIB-IIIA; FIBRINOGEN RECEPTOR; SURFACE EXPRESSION;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Agriculture,Biology & Environmental Sciences
Life Sciences
Citazioni:
141
Recensione:
Indirizzi per estratti:
Indirizzo: Boudreaux, MK Auburn Univ, Coll Vet Med, Dept Pathobiol, 166 Green Hall, Auburn, AL 36849 USA Auburn Univ 166 Green Hall Auburn AL USA 36849 AL 36849 USA
Citazione:
M.K. Boudreaux e D.L. Lipscomb, "Clinical, biochemical, and molecular aspects of Glanzmann's thrombastheniain humans and dogs", VET PATH, 38(3), 2001, pp. 249-260

Abstract

Glanzmann's thrombasthenia (GT) is an inherited, intrinsic platelet function defect that involves the platelet glycoprotein complex IIb-IIIa, also known as the fibrinogen receptor and the integrin a,,P,. The defect was originally described by Dr. Glanzmann in humans in 1918 as a bleeding disorder that differed clinically from other known coagulopathies. Over the decades that followed, researchers determined the biochemical and molecular basis for the disease in humans. Otterhounds with thrombasthenic thrombopathia, described in the 1960s, were the only animal model that closely resembled the disease described in humans until 1996. At that time, a Great Pyrenees dog was identified with unequivocal clinical and biochemical features of Type IGT. The cDNA encoding for glycoproteins IIb and IIIa were sequenced in normal dogs in 1999, allowing for identification of specific mutations causingType I GT in both Otterhounds and Great Pyrenees dogs. Knowing the molecular basis for Type I GT in dogs as well as the cDNA sequences in normal dogsshould enhance the understanding of structure/function relationships of the oi,,,P, integrin and provide an excellent animal model for studies aimed at correction of GT in humans. The following review focuses on the structure and function of this platelet receptor and reviews the molecular biochemical, and clinical aspects of Glanzmann's thrombasthmia in humans and dogs.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 12:44:45