Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
2008-3-4
pubmed:abstractText
In humans, hereditary inactivation of either p22(phox) or gp91(phox) leads to chronic granulomatous disease (CGD), a severe immune disorder characterized by the inability of phagocytes to produce bacteria-destroying ROS. Heterodimers of p22(phox) and gp91(phox) proteins constitute the superoxide-producing cytochrome core of the phagocyte NADPH oxidase. In this study, we identified the nmf333 mouse strain as what we believe to be the first animal model of p22(phox) deficiency. Characterization of nmf333 mice revealed that deletion of p22(phox) inactivated not only the phagocyte NADPH oxidase, but also a second cytochrome in the inner ear epithelium. As a consequence, mice of the nmf333 strain exhibit a compound phenotype consisting of both a CGD-like immune defect and a balance disorder caused by the aberrant development of gravity-sensing organs. Thus, in addition to identifying a model of p22(phox)-dependent immune deficiency, our study indicates that a clinically identifiable patient population with an otherwise cryptic loss of gravity-sensor function may exist. Thus, p22(phox) represents a shared and essential component of at least 2 superoxide-producing cytochromes with entirely different biological functions. The site of p22(phox) expression in the inner ear leads us to propose what we believe to be a novel mechanism for the control of vestibular organogenesis.
pubmed:grant
pubmed:commentsCorrections
http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-10432282, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-10511626, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-10556201, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-11152663, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-11710459, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-12110737, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-12473664, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-12651873, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-12657628, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-12673252, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-12716910, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-12824283, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-14690057, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15014044, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15039755, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15073033, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15181005, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15322091, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15326186, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15561463, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15749893, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15824103, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15857905, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15927447, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-15994299, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-16230485, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-16246966, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-16386251, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-16431374, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-16529728, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-16615898, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-17082494, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-17133097, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-17140397, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-17200157, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-17237347, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-17300776, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-17670895, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-1974155, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-2993312, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-7219526, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-7650482, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-7719350, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-8634451, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-9334224, http://linkedlifedata.com/resource/pubmed/commentcorrection/18292807-9565037
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
0021-9738
pubmed:author
pubmed:issnType
Print
pubmed:volume
118
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1176-85
pubmed:dateRevised
2010-12-3
pubmed:meshHeading
More...