| Distribution of type IV collagen in the cochlea in Alport syndrome. | |
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MedLine Citation:
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PMID: 16301374 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To determine the distribution of alpha1, alpha3, and alpha5 chains of type IV collagen in the cochlea in Alport syndrome. DESIGN: Case-control study. PATIENTS: Two patients with sensorineural hearing loss due to Alport syndrome. Both patients had known mutations in the COL4A5 gene. MAIN OUTCOME MEASURES: Immunostaining was used to study the distribution of type IV collagen (alpha1, alpha3, and alpha5 chains) within the cochlea. Immunostaining was also performed in the cochlear tissues of an unaffected individual used as a control. RESULTS: In the control ear, alpha1 staining was observed in the basement membrane overlying the basilar membrane, in the basement membrane of cochlear blood vessels and Schwann cells, and within the spiral limbus. In the control ear, we also observed strong staining for alpha3 and alpha5 chains in the basement membrane overlying the basilar membrane and within the spiral ligament. In both cases with Alport syndrome, no immunostaining was observed for alpha3 or alpha5 chains within the cochlea, whereas alpha1 staining was present in locations similar to that seen in the control ear. CONCLUSIONS: The results indicate that isotype switching does not occur within the cochlea in Alport syndrome. The results are also consistent with the hypothesis that the sensorineural hearing loss in Alport syndrome may be due to alterations in cochlear micromechanics and/or dysfunction of the spiral ligament. |
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Authors:
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Andreas F Zehnder; Joe C Adams; Peter A Santi; Arthur G Kristiansen; Chitsuda Wacharasindhu; Sabine Mann; Raghu Kalluri; Martin C Gregory; Clifford E Kashtan; Saumil N Merchant |
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Publication Detail:
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Type: Case Reports; Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Archives of otolaryngology--head & neck surgery Volume: 131 ISSN: 0886-4470 ISO Abbreviation: Arch. Otolaryngol. Head Neck Surg. Publication Date: 2005 Nov |
Date Detail:
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Created Date: 2005-11-22 Completed Date: 2005-12-28 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8603209 Medline TA: Arch Otolaryngol Head Neck Surg Country: United States |
Other Details:
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Languages: eng Pagination: 1007-13 Citation Subset: AIM; IM |
Affiliation:
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Ear, Nose, and Throat Department, University Hospital Basel, Basel, Switzerland. |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Basement Membrane / metabolism Case-Control Studies Cochlea / blood supply, cytology, metabolism* Collagen Type IV / genetics, metabolism* Coloring Agents Eosine Yellowish-(YS) Female Fluorescent Dyes Hearing Loss, Sensorineural / etiology, metabolism Hematoxylin Humans Male Microscopy, Polarization Middle Aged Mutation / genetics Nephritis, Hereditary / complications, metabolism* Schwann Cells / metabolism |
| Chemical | |
Reg. No./Substance:
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0/COL4A5 protein, human; 0/Collagen Type IV; 0/Coloring Agents; 0/Fluorescent Dyes; 17372-87-1/Eosine Yellowish-(YS); 517-28-2/Hematoxylin |
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