Loading…
A Novel X-Linked Disorder of Immune Deficiency and Hypohidrotic Ectodermal Dysplasia Is Allelic to Incontinentia Pigmenti and Due to Mutations in IKK-gamma ( NEMO)
Hypohidrotic ectodermal dysplasia (HED), a congenital disorder of teeth, hair, and eccrine sweat glands, is usually inherited as an X-linked recessive trait, although rarer autosomal dominant and recessive forms exist. We have studied males from four families with HED and immunodeficiency (HED-ID),...
Saved in:
Published in: | American journal of human genetics 2000-12, Vol.67 (6), p.1555-1562 |
---|---|
Main Authors: | , , , , , , , , , , |
Format: | Article |
Language: | English |
Subjects: | |
Citations: | Items that this one cites Items that cite this one |
Online Access: | Get full text |
Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
cited_by | cdi_FETCH-LOGICAL-c497t-79f4d7dc026f2e70338de23f5c92f00fb45e5e2889f311e4655a4b19eb1122af3 |
---|---|
cites | cdi_FETCH-LOGICAL-c497t-79f4d7dc026f2e70338de23f5c92f00fb45e5e2889f311e4655a4b19eb1122af3 |
container_end_page | 1562 |
container_issue | 6 |
container_start_page | 1555 |
container_title | American journal of human genetics |
container_volume | 67 |
creator | Zonana, Jonathan Elder, Melissa E. Schneider, Lynda C. Orlow, Seth J. Moss, Celia Golabi, Mahin Shapira, Stuart K. Farndon, Peter A. Wara, Diane W. Emmal, Stephanie A. Ferguson, Betsy M. |
description | Hypohidrotic ectodermal dysplasia (HED), a congenital disorder of teeth, hair, and eccrine sweat glands, is usually inherited as an X-linked recessive trait, although rarer autosomal dominant and recessive forms exist. We have studied males from four families with HED and immunodeficiency (HED-ID), in which the disorder segregates as an X-linked recessive trait. Affected males manifest dysgammaglobulinemia and, despite therapy, have significant morbidity and mortality from recurrent infections. Recently, mutations in
IKK-gamma (
NEMO) have been shown to cause familial incontinentia pigmenti (IP). Unlike HED-ID, IP affects females and, with few exceptions, causes male prenatal lethality. IKK-gamma is required for the activation of the transcription factor known as “nuclear factor kappa B” and plays an important role in T and B cell function. We hypothesize that “milder” mutations at this locus may cause HED-ID. In all four families, sequence analysis reveals exon 10 mutations affecting the carboxy-terminal end of the IKK-gamma protein, a domain believed to connect the IKK signalsome complex to upstream activators. The findings define a new X-linked recessive immunodeficiency syndrome, distinct from other types of HED and immunodeficiency syndromes. The data provide further evidence that the development of ectodermal appendages is mediated through a tumor necrosis factor/tumor necrosis factor receptor–like signaling pathway, with the IKK signalsome complex playing a significant role. |
doi_str_mv | 10.1086/316914 |
format | article |
fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1287930</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S0002929707632236</els_id><sourcerecordid>72418555</sourcerecordid><originalsourceid>FETCH-LOGICAL-c497t-79f4d7dc026f2e70338de23f5c92f00fb45e5e2889f311e4655a4b19eb1122af3</originalsourceid><addsrcrecordid>eNpdkcFuEzEQhi0EoqHAIyBLSAgOC7Z3vd69IEVNSqOmLQeQuFmOPU4NXjvYu5HyPLxoN03UApeZkebT_4_mR-g1JR8paepPJa1bWj1BE8pLUdQ14U_RhBDCipa14gS9yPknIZQ2pHyOTigllRBcTNCfKb6OW_D4R7F04RcYPHM5JgMJR4sXXTcEwDOwTjsIeodVMPhit4m3zqTYO43nuo8j3SmPZ7u88So7hRcZT70HP-77iBdBx9C7AGNR-Ktbd_vpXmo2wJ64GnrVuxgydgEvLi-Lteo6hd_j6_nVzYeX6JlVPsOrYz9F38_n384uiuXNl8XZdFnoqhV9IVpbGWE0YbVlIEhZNgZYablumSXErioOHFjTtLakFKqac1WtaAsrShlTtjxFnw-6m2HVgdHjlUl5uUmuU2kno3Ly301wt3Idt5KyRrQlGQXeHQVS_D1A7mXnsgbvVYA4ZClYRRvO-SOoU8w5gX0woUTu85SHPEfwzd8nPWLHAEfg7RFQWStvkwra5QeuYTWv9n7kQMH4vq2DJPN9nmBcAt1LE93_zncC4Lf4</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>72418555</pqid></control><display><type>article</type><title>A Novel X-Linked Disorder of Immune Deficiency and Hypohidrotic Ectodermal Dysplasia Is Allelic to Incontinentia Pigmenti and Due to Mutations in IKK-gamma ( NEMO)</title><source>BACON - Elsevier - GLOBAL_SCIENCEDIRECT-OPENACCESS</source><source>PubMed Central</source><creator>Zonana, Jonathan ; Elder, Melissa E. ; Schneider, Lynda C. ; Orlow, Seth J. ; Moss, Celia ; Golabi, Mahin ; Shapira, Stuart K. ; Farndon, Peter A. ; Wara, Diane W. ; Emmal, Stephanie A. ; Ferguson, Betsy M.</creator><creatorcontrib>Zonana, Jonathan ; Elder, Melissa E. ; Schneider, Lynda C. ; Orlow, Seth J. ; Moss, Celia ; Golabi, Mahin ; Shapira, Stuart K. ; Farndon, Peter A. ; Wara, Diane W. ; Emmal, Stephanie A. ; Ferguson, Betsy M.</creatorcontrib><description>Hypohidrotic ectodermal dysplasia (HED), a congenital disorder of teeth, hair, and eccrine sweat glands, is usually inherited as an X-linked recessive trait, although rarer autosomal dominant and recessive forms exist. We have studied males from four families with HED and immunodeficiency (HED-ID), in which the disorder segregates as an X-linked recessive trait. Affected males manifest dysgammaglobulinemia and, despite therapy, have significant morbidity and mortality from recurrent infections. Recently, mutations in
IKK-gamma (
NEMO) have been shown to cause familial incontinentia pigmenti (IP). Unlike HED-ID, IP affects females and, with few exceptions, causes male prenatal lethality. IKK-gamma is required for the activation of the transcription factor known as “nuclear factor kappa B” and plays an important role in T and B cell function. We hypothesize that “milder” mutations at this locus may cause HED-ID. In all four families, sequence analysis reveals exon 10 mutations affecting the carboxy-terminal end of the IKK-gamma protein, a domain believed to connect the IKK signalsome complex to upstream activators. The findings define a new X-linked recessive immunodeficiency syndrome, distinct from other types of HED and immunodeficiency syndromes. The data provide further evidence that the development of ectodermal appendages is mediated through a tumor necrosis factor/tumor necrosis factor receptor–like signaling pathway, with the IKK signalsome complex playing a significant role.</description><identifier>ISSN: 0002-9297</identifier><identifier>EISSN: 1537-6605</identifier><identifier>DOI: 10.1086/316914</identifier><identifier>PMID: 11047757</identifier><identifier>CODEN: AJHGAG</identifier><language>eng</language><publisher>Chicago, IL: Elsevier Inc</publisher><subject>Adolescent ; Alleles ; Base Sequence ; Biological and medical sciences ; Child ; Child, Preschool ; Complex syndromes ; DNA Mutational Analysis ; Ectodermal Dysplasia - complications ; Ectodermal Dysplasia - genetics ; Exons - genetics ; Female ; Genes, Recessive - genetics ; Genetic Linkage - genetics ; Humans ; I-kappa B Kinase ; Immunologic Deficiency Syndromes - complications ; Immunologic Deficiency Syndromes - genetics ; Incontinentia Pigmenti - genetics ; Infant ; Infant, Newborn ; Male ; Medical genetics ; Medical sciences ; Mutation - genetics ; NF-kappa B - physiology ; Pedigree ; Protein Structure, Tertiary ; Protein-Serine-Threonine Kinases - chemistry ; Protein-Serine-Threonine Kinases - genetics ; X Chromosome - genetics</subject><ispartof>American journal of human genetics, 2000-12, Vol.67 (6), p.1555-1562</ispartof><rights>2000 The American Society of Human Genetics</rights><rights>2001 INIST-CNRS</rights><rights>2000 by The American Society of Human Genetics. All rights reserved. 2000</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c497t-79f4d7dc026f2e70338de23f5c92f00fb45e5e2889f311e4655a4b19eb1122af3</citedby><cites>FETCH-LOGICAL-c497t-79f4d7dc026f2e70338de23f5c92f00fb45e5e2889f311e4655a4b19eb1122af3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1287930/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1287930/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,314,727,780,784,885,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=826545$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11047757$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Zonana, Jonathan</creatorcontrib><creatorcontrib>Elder, Melissa E.</creatorcontrib><creatorcontrib>Schneider, Lynda C.</creatorcontrib><creatorcontrib>Orlow, Seth J.</creatorcontrib><creatorcontrib>Moss, Celia</creatorcontrib><creatorcontrib>Golabi, Mahin</creatorcontrib><creatorcontrib>Shapira, Stuart K.</creatorcontrib><creatorcontrib>Farndon, Peter A.</creatorcontrib><creatorcontrib>Wara, Diane W.</creatorcontrib><creatorcontrib>Emmal, Stephanie A.</creatorcontrib><creatorcontrib>Ferguson, Betsy M.</creatorcontrib><title>A Novel X-Linked Disorder of Immune Deficiency and Hypohidrotic Ectodermal Dysplasia Is Allelic to Incontinentia Pigmenti and Due to Mutations in IKK-gamma ( NEMO)</title><title>American journal of human genetics</title><addtitle>Am J Hum Genet</addtitle><description>Hypohidrotic ectodermal dysplasia (HED), a congenital disorder of teeth, hair, and eccrine sweat glands, is usually inherited as an X-linked recessive trait, although rarer autosomal dominant and recessive forms exist. We have studied males from four families with HED and immunodeficiency (HED-ID), in which the disorder segregates as an X-linked recessive trait. Affected males manifest dysgammaglobulinemia and, despite therapy, have significant morbidity and mortality from recurrent infections. Recently, mutations in
IKK-gamma (
NEMO) have been shown to cause familial incontinentia pigmenti (IP). Unlike HED-ID, IP affects females and, with few exceptions, causes male prenatal lethality. IKK-gamma is required for the activation of the transcription factor known as “nuclear factor kappa B” and plays an important role in T and B cell function. We hypothesize that “milder” mutations at this locus may cause HED-ID. In all four families, sequence analysis reveals exon 10 mutations affecting the carboxy-terminal end of the IKK-gamma protein, a domain believed to connect the IKK signalsome complex to upstream activators. The findings define a new X-linked recessive immunodeficiency syndrome, distinct from other types of HED and immunodeficiency syndromes. The data provide further evidence that the development of ectodermal appendages is mediated through a tumor necrosis factor/tumor necrosis factor receptor–like signaling pathway, with the IKK signalsome complex playing a significant role.</description><subject>Adolescent</subject><subject>Alleles</subject><subject>Base Sequence</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Complex syndromes</subject><subject>DNA Mutational Analysis</subject><subject>Ectodermal Dysplasia - complications</subject><subject>Ectodermal Dysplasia - genetics</subject><subject>Exons - genetics</subject><subject>Female</subject><subject>Genes, Recessive - genetics</subject><subject>Genetic Linkage - genetics</subject><subject>Humans</subject><subject>I-kappa B Kinase</subject><subject>Immunologic Deficiency Syndromes - complications</subject><subject>Immunologic Deficiency Syndromes - genetics</subject><subject>Incontinentia Pigmenti - genetics</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Male</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>Mutation - genetics</subject><subject>NF-kappa B - physiology</subject><subject>Pedigree</subject><subject>Protein Structure, Tertiary</subject><subject>Protein-Serine-Threonine Kinases - chemistry</subject><subject>Protein-Serine-Threonine Kinases - genetics</subject><subject>X Chromosome - genetics</subject><issn>0002-9297</issn><issn>1537-6605</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2000</creationdate><recordtype>article</recordtype><recordid>eNpdkcFuEzEQhi0EoqHAIyBLSAgOC7Z3vd69IEVNSqOmLQeQuFmOPU4NXjvYu5HyPLxoN03UApeZkebT_4_mR-g1JR8paepPJa1bWj1BE8pLUdQ14U_RhBDCipa14gS9yPknIZQ2pHyOTigllRBcTNCfKb6OW_D4R7F04RcYPHM5JgMJR4sXXTcEwDOwTjsIeodVMPhit4m3zqTYO43nuo8j3SmPZ7u88So7hRcZT70HP-77iBdBx9C7AGNR-Ktbd_vpXmo2wJ64GnrVuxgydgEvLi-Lteo6hd_j6_nVzYeX6JlVPsOrYz9F38_n384uiuXNl8XZdFnoqhV9IVpbGWE0YbVlIEhZNgZYablumSXErioOHFjTtLakFKqac1WtaAsrShlTtjxFnw-6m2HVgdHjlUl5uUmuU2kno3Ly301wt3Idt5KyRrQlGQXeHQVS_D1A7mXnsgbvVYA4ZClYRRvO-SOoU8w5gX0woUTu85SHPEfwzd8nPWLHAEfg7RFQWStvkwra5QeuYTWv9n7kQMH4vq2DJPN9nmBcAt1LE93_zncC4Lf4</recordid><startdate>20001201</startdate><enddate>20001201</enddate><creator>Zonana, Jonathan</creator><creator>Elder, Melissa E.</creator><creator>Schneider, Lynda C.</creator><creator>Orlow, Seth J.</creator><creator>Moss, Celia</creator><creator>Golabi, Mahin</creator><creator>Shapira, Stuart K.</creator><creator>Farndon, Peter A.</creator><creator>Wara, Diane W.</creator><creator>Emmal, Stephanie A.</creator><creator>Ferguson, Betsy M.</creator><general>Elsevier Inc</general><general>University of Chicago Press</general><general>The American Society of Human Genetics</general><scope>6I.</scope><scope>AAFTH</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20001201</creationdate><title>A Novel X-Linked Disorder of Immune Deficiency and Hypohidrotic Ectodermal Dysplasia Is Allelic to Incontinentia Pigmenti and Due to Mutations in IKK-gamma ( NEMO)</title><author>Zonana, Jonathan ; Elder, Melissa E. ; Schneider, Lynda C. ; Orlow, Seth J. ; Moss, Celia ; Golabi, Mahin ; Shapira, Stuart K. ; Farndon, Peter A. ; Wara, Diane W. ; Emmal, Stephanie A. ; Ferguson, Betsy M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c497t-79f4d7dc026f2e70338de23f5c92f00fb45e5e2889f311e4655a4b19eb1122af3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2000</creationdate><topic>Adolescent</topic><topic>Alleles</topic><topic>Base Sequence</topic><topic>Biological and medical sciences</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Complex syndromes</topic><topic>DNA Mutational Analysis</topic><topic>Ectodermal Dysplasia - complications</topic><topic>Ectodermal Dysplasia - genetics</topic><topic>Exons - genetics</topic><topic>Female</topic><topic>Genes, Recessive - genetics</topic><topic>Genetic Linkage - genetics</topic><topic>Humans</topic><topic>I-kappa B Kinase</topic><topic>Immunologic Deficiency Syndromes - complications</topic><topic>Immunologic Deficiency Syndromes - genetics</topic><topic>Incontinentia Pigmenti - genetics</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Male</topic><topic>Medical genetics</topic><topic>Medical sciences</topic><topic>Mutation - genetics</topic><topic>NF-kappa B - physiology</topic><topic>Pedigree</topic><topic>Protein Structure, Tertiary</topic><topic>Protein-Serine-Threonine Kinases - chemistry</topic><topic>Protein-Serine-Threonine Kinases - genetics</topic><topic>X Chromosome - genetics</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Zonana, Jonathan</creatorcontrib><creatorcontrib>Elder, Melissa E.</creatorcontrib><creatorcontrib>Schneider, Lynda C.</creatorcontrib><creatorcontrib>Orlow, Seth J.</creatorcontrib><creatorcontrib>Moss, Celia</creatorcontrib><creatorcontrib>Golabi, Mahin</creatorcontrib><creatorcontrib>Shapira, Stuart K.</creatorcontrib><creatorcontrib>Farndon, Peter A.</creatorcontrib><creatorcontrib>Wara, Diane W.</creatorcontrib><creatorcontrib>Emmal, Stephanie A.</creatorcontrib><creatorcontrib>Ferguson, Betsy M.</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>American journal of human genetics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Zonana, Jonathan</au><au>Elder, Melissa E.</au><au>Schneider, Lynda C.</au><au>Orlow, Seth J.</au><au>Moss, Celia</au><au>Golabi, Mahin</au><au>Shapira, Stuart K.</au><au>Farndon, Peter A.</au><au>Wara, Diane W.</au><au>Emmal, Stephanie A.</au><au>Ferguson, Betsy M.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A Novel X-Linked Disorder of Immune Deficiency and Hypohidrotic Ectodermal Dysplasia Is Allelic to Incontinentia Pigmenti and Due to Mutations in IKK-gamma ( NEMO)</atitle><jtitle>American journal of human genetics</jtitle><addtitle>Am J Hum Genet</addtitle><date>2000-12-01</date><risdate>2000</risdate><volume>67</volume><issue>6</issue><spage>1555</spage><epage>1562</epage><pages>1555-1562</pages><issn>0002-9297</issn><eissn>1537-6605</eissn><coden>AJHGAG</coden><abstract>Hypohidrotic ectodermal dysplasia (HED), a congenital disorder of teeth, hair, and eccrine sweat glands, is usually inherited as an X-linked recessive trait, although rarer autosomal dominant and recessive forms exist. We have studied males from four families with HED and immunodeficiency (HED-ID), in which the disorder segregates as an X-linked recessive trait. Affected males manifest dysgammaglobulinemia and, despite therapy, have significant morbidity and mortality from recurrent infections. Recently, mutations in
IKK-gamma (
NEMO) have been shown to cause familial incontinentia pigmenti (IP). Unlike HED-ID, IP affects females and, with few exceptions, causes male prenatal lethality. IKK-gamma is required for the activation of the transcription factor known as “nuclear factor kappa B” and plays an important role in T and B cell function. We hypothesize that “milder” mutations at this locus may cause HED-ID. In all four families, sequence analysis reveals exon 10 mutations affecting the carboxy-terminal end of the IKK-gamma protein, a domain believed to connect the IKK signalsome complex to upstream activators. The findings define a new X-linked recessive immunodeficiency syndrome, distinct from other types of HED and immunodeficiency syndromes. The data provide further evidence that the development of ectodermal appendages is mediated through a tumor necrosis factor/tumor necrosis factor receptor–like signaling pathway, with the IKK signalsome complex playing a significant role.</abstract><cop>Chicago, IL</cop><pub>Elsevier Inc</pub><pmid>11047757</pmid><doi>10.1086/316914</doi><tpages>8</tpages><oa>free_for_read</oa></addata></record> |
fulltext | fulltext |
identifier | ISSN: 0002-9297 |
ispartof | American journal of human genetics, 2000-12, Vol.67 (6), p.1555-1562 |
issn | 0002-9297 1537-6605 |
language | eng |
recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1287930 |
source | BACON - Elsevier - GLOBAL_SCIENCEDIRECT-OPENACCESS; PubMed Central |
subjects | Adolescent Alleles Base Sequence Biological and medical sciences Child Child, Preschool Complex syndromes DNA Mutational Analysis Ectodermal Dysplasia - complications Ectodermal Dysplasia - genetics Exons - genetics Female Genes, Recessive - genetics Genetic Linkage - genetics Humans I-kappa B Kinase Immunologic Deficiency Syndromes - complications Immunologic Deficiency Syndromes - genetics Incontinentia Pigmenti - genetics Infant Infant, Newborn Male Medical genetics Medical sciences Mutation - genetics NF-kappa B - physiology Pedigree Protein Structure, Tertiary Protein-Serine-Threonine Kinases - chemistry Protein-Serine-Threonine Kinases - genetics X Chromosome - genetics |
title | A Novel X-Linked Disorder of Immune Deficiency and Hypohidrotic Ectodermal Dysplasia Is Allelic to Incontinentia Pigmenti and Due to Mutations in IKK-gamma ( NEMO) |
url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-24T22%3A31%3A05IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=A%20Novel%20X-Linked%20Disorder%20of%20Immune%20Deficiency%20and%20Hypohidrotic%20Ectodermal%20Dysplasia%20Is%20Allelic%20to%20Incontinentia%20Pigmenti%20and%20Due%20to%20Mutations%20in%20IKK-gamma%20(%20NEMO)&rft.jtitle=American%20journal%20of%20human%20genetics&rft.au=Zonana,%20Jonathan&rft.date=2000-12-01&rft.volume=67&rft.issue=6&rft.spage=1555&rft.epage=1562&rft.pages=1555-1562&rft.issn=0002-9297&rft.eissn=1537-6605&rft.coden=AJHGAG&rft_id=info:doi/10.1086/316914&rft_dat=%3Cproquest_pubme%3E72418555%3C/proquest_pubme%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c497t-79f4d7dc026f2e70338de23f5c92f00fb45e5e2889f311e4655a4b19eb1122af3%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=72418555&rft_id=info:pmid/11047757&rfr_iscdi=true |