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Titolo:
Anosmin-1 is a regionally restricted component of basement membranes and interstitial matrices during organogenesis: Implications for the developmental anomalies of X chromosome-linked Kallmann syndrome
Autore:
Hardelin, JP; Julliard, AK; Moniot, B; Soussi-Yanicostas, N; Verney, C; Schwanzel-Fukuda, M; Lievre, CA; Petit, C;
Indirizzi:
InstsPasteur, Unite Genet Deficits Sensoriels, CNRS, URA 1968, F-75724 Pari Inst Pasteur Paris France 15 ts Sensoriels, CNRS, URA 1968, F-75724 Pari Univleurbanne,NRS, UPRESA 5020, Lab Neurosci & Syst Sensoriels, F-69622 Vil Univ Lyon 1 Villeurbanne France F-69622 i & Syst Sensoriels, F-69622 Vil CNRS, IGH, UPR 1142, Montpellier, France CNRS Montpellier FranceCNRS, IGH, UPR 1142, Montpellier, France Hop La Pitie Salpetriere, INSERM, U106, Paris, France Hop La Pitie Salpetriere Paris France iere, INSERM, U106, Paris, France SUNY Hlth Sci Ctr, Dept Anat & Cell Biol, Brooklyn, NY 11203 USA SUNY HlthSci Ctr Brooklyn NY USA 11203 Cell Biol, Brooklyn, NY 11203 USA CNRS, ER 1794, Fac Med, Limoges, France CNRS Limoges FranceCNRS, ER 1794, Fac Med, Limoges, France
Titolo Testata:
DEVELOPMENTAL DYNAMICS
fascicolo: 1, volume: 215, anno: 1999,
pagine: 26 - 44
SICI:
1058-8388(199905)215:1<26:AIARRC>2.0.ZU;2-8
Fonte:
ISI
Lingua:
ENG
Soggetto:
HORMONE-RELEASING HORMONE; NEURAL CELL-ADHESION; AMINO-ACID-SEQUENCE; LAMININ-A-CHAIN; EXTRACELLULAR-MATRIX; MESSENGER-RNA; LHRH NEURONS; BRANCHING MORPHOGENESIS; SYNDROME GENE; OLFACTORY EPITHELIUM;
Keywords:
human embryonic development; kidney organogenesis; thalamus; olfactory receptor neurons; motor neurons; nervus terminalis; GnRH-synthesizing neurons; olfactory bulbs; retina; inner ear; extracellular matrix; laminin; KAL-1; electron microscopy;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
75
Recensione:
Indirizzi per estratti:
Indirizzo: Hardelin, JP Instux,steur, Unite Genet Deficits Sensoriels, CNRS, URA 1968, 25 Rue Dr Ro Inst Pasteur 25 Rue Dr Roux Paris France 15 68, 25 Rue Dr Ro
Citazione:
J.P. Hardelin et al., "Anosmin-1 is a regionally restricted component of basement membranes and interstitial matrices during organogenesis: Implications for the developmental anomalies of X chromosome-linked Kallmann syndrome", DEV DYNAM, 215(1), 1999, pp. 26-44

Abstract

Kallmann syndrome is a developmental disease characterized by gonadotropin-releasing hormone (GnRH) deficiency and olfactory bulb hypoplasia. The gene underlying the X chromosome-linked form, KAL-1, has been identified for several years, yet the pathogenesis of the disease is not understood. By immunohistofluorescence and immunoelectron microscopy, we establish that the KAL-1 encoded protein, anosmin-1, is a transient and regionally restricted component of extracellular matrices during organogenesis in man. Anosmin-1 was detected in the basement membranes and/or interstitial matrices of various structures including bronchial tubes, mesonephric tubules and duct, branches of the ureteric bud, muscular walls of the digestive tract and larger blood vessels, precartilaginous models of skeletal pieces, muscle tendons, head mesenchymes, inner ear, and forebrain subregions. Our results suggest that this protein acts as a local, rather than a long-range, cue during organogenesis. In the olfactory system, anosmin-1 was detected from week 5 onward. The protein was restricted to the olfactory bulb presumptive region and later, to the primitive olfactory bulbs. We therefore suggest that the genetic defect underlying X-linked Kallmann syndrome disrupts the terminal navigation of the early olfactory axons or directly affects the initial stepsof olfactory bulb differentiation. The mechanism of the GnRH deficiency isalso discussed, relying on the evidence that anosmin-1 is present in the medial walls of the primitive cerebral hemispheres, along the rostro-caudal migratory pathway of the GnRH-synthesizing neurons, at 6 weeks. Finally, the present results strongly suggest that the renal aplasia observed in aboutone third of the affected individuals results from primary failure of the collecting duct system. (C) 1999 Wiley-Liss,Inc.

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Documento generato il 07/04/20 alle ore 23:07:53