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BusinessTGloI〕ｈｏｌｌｃ：＋81-282-87-2171,ＦＡＸ：÷81-282-86-`1940、
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ｍＲＮＡｉｎｍｕｓcleinapatientwith
mcrosin-negativecongenitalmuscular
dystrophy､Ｍｕｓｃｌｅ＆Norvel995；１８：10271030.

10）Ｋ()rOmatsuS，Kobayashi（)，FukushimaN，ｃｔａｌ：Ｎｏｎ－ＦｕｋｕｙａｍａＬｙｐｅｍｅｒｏｓｉｎ－ｐ()sitivecongenitalmusculardystrophy
wiLhdelayedmusclefibertypedifferentiation：acasereport［Japanese]、NotollaiLaLsu［Braｉｎ＆Development］1995；
２７：309-314.

11）大谷宜{''１，吉I1Yii毅，三池脚久，他：メロシン（ラミニンＭ）欠孤症の同胞例．脳と発達１９９５；２７：SupplcS26al2）瓜満，佐々木照子，西１１｜直樹，他：多数のネマリン小休を伴ったメロシン陰性先天性筋ジストロフィーの１例．脳と発達１９９６；２８：SupplS215、
13）本ｌＩｌ１彰，石井玲子，小林治，他：メロ
シン陰性先天性筋ジストロフィ_の１例．
日本小児科学会誌1998；１０２：522.
1`1）Ｉ)ubowitzV：Workshopreport、２２ndEuropoanNeuromuscularOonsortium-Spon‐sorcdworkshoponcongeniLalmuscular
dystrophy、Ncuromuscl994；Disord4：75-81.

4）TomeFMEvangelistaT，LeclercA，ｅｔ15）VuolteenahoR，NissinenM，SainioK，ｅｔａｌ：ＨｕｍａｎｌａｍｉｎｉｎＭｃｈａｉｎ（merosin)：

morosindeliciency、ＣｏｍｐＬｅｓＲｅｎｄｕｓｄｅｌ
ＡｃａｄｅｍｉｅｄｅｓＳｃｉencos-Serielii，SciencesdelaViel994；３１７：351-357.

ｃ()ｍｐｌｅｔｅｐｒｉｍａｒｙｓｔｒｕｃｔｕ１.c，ｃｈｒｏｍｏｓｏ－ａｌ：CongenitalmusculardvsLropllywiLh5）PhilpotJ，ＳｅｗｒｙＣ，Penn()ｃｋＪ，ｅｔａｌ：（jlinica」ｐｈｅnotypcincoI1geniLa］ｍｕｓｃｕ‐
ｌａｒｄｙｓｔｒｏｐｈｙ：ｃｏｒｌ､olaLionwithcxpres‐sionofmerosininskolotalmuscle・Ｎｅｕ‐romuscularDisordersl995；５：301-305.

6）KobavashiO，HayashiY，ArahataK，ｅｔ

ａｌ：Congenitalmusculardvstrophy：Clini‐

5８

ｍａｌａｓｓｉｇｎｍｅｎｔ，ａｎｄｃｘｐｒｅｓｓｉｏｎｏｆｔｈｅＭ
ａｎｄＡｃｈａｉｎｉｎｈｕｍａｎｆｅｔａｌｔｉｓｓｕｃｓ・Ｊｏｕｒ－ｎａｌ（)fCellBiologyl994；124：381-394.
16）VainzofM，ＭａｒｉｃＳＫ，ＲｃｏｄＵＣ，ｅｔａｌ：
Ｉ)eficiencyofmerosin（ｌａｍｉｎｉｎＭｏｒａｌ‐
ｐｈａ２）incongenitalmusculardystrophy
associatedwithcercbralwhitemaLter

alterations，NeuropodiaLricsl995，２６：２９３－
２９７．

ＶｏＬｌ４Ｎｏ､3,1998203

17）TrevisanCP，MaI･tinelloF，FerruzzaE，ｅｔａｌ：Brainaltorati()Ｉ１ｓｉｌ１ＬｈｅｃｌａｓｓｉｃａｌｆｏｒｍｏｆｃｏｎｇｅｎｉＬａｌｍｕｓｃｕｌａｒ〔lystroplly・Clinicalandncuroimaging「ｏｌｌｏｗ－ｕｐｏｆ
ｌ２ｃａｓｅｓａｎｄｃ()rrolationwithLheexpres‐ｓｉｏｎｏｆｍｅｒｏｓｉｎｉｎｍｕｓｃｌ()，（】ｈｉｌｄｓＸｅｒ‐

ｖｏｕｓＳｙｓｔｅｍｌＩ)96；１２：６()'1-610.

18）ｖａｎｄｏｒＫｎａａｐＭＳ，ＳｍｉｔｌＭ，BarthＰＧ，ｅｔａＩ：ＭａｇｎｅＬｉｃｒｅｓｏｎａｎｃｅｉｍａｇｉｎｇｉｎ
ｃｌａＳＳｉ｢ication（)fcongenitalmuscular
dystrophiesｗｉＬｈｌ)ｒａｉｎａ１)、()rmalities，ＡｎｎａｌｓｏｌＮｏｕｒｏｌｏｇｙｌ９９７ｉ４２：50-59.

19）MerculiE，Ｉ)ubowiLzL，Beral･dinelliA，ｅｔａｌ：Ｍｉｎｏｒｎｅｕｒｏｌｏｇｉｃａｌａｎｄｐｅｒｃｅｐ－
ｔｕｏ－ｍ〔)tordeficitsinchildr〔)、ｗｉｔｈｃｏｎ－

ｇｅｎｉＬａｌｍｕｓｃｕｌａｒｄｙｓｔｌ･ｏｐｈｙ：ｃｏｒｒｅｌａｔｉｏｎ
ｗｉｔｈｂｒａｉｎＭＲＩｃｈａｎｇｅｓ．Ｎ(1uropediatrics
l995；２６：１５６－１６２．

２０）ReedITC，ＭａｒｉｅＳＫ，VainzoflI，ｅｔａｌ：
CongenitalmuIx1ulardystl･()phywith
cerobralwhitemattcrhypodensiLy．Ｃor‐
relaLionofclinical「eaturesandmerosin

deficiency・Braｉｎ＆Dovelopmentl996；
１８：53-58.

21）Lamel．Ｓ，Cal･lierR，PinardJ，ｅｔａｌ：
CongenitalmuHculardysh･()phy：ｕｓｅｏｆ
ｂｒａｉｎＭＲｉｍａｇｉｎｇｆｉｎｄｉｎｇｓＬｏｐredictmerosin（leficiency・Radiologyl998；206：
811-816.

22）SunadaY，lddgarTS，ＬｏｔｚＢＩ〕，ｏｔａｌ：
Merosin-nogativccongenitalmuscular
dystrophyassociaLedwiLhextensivebrain
abnormalities、Neurologyl995；、15：20842089.

23）Ｍｅ1℃ul･ｉｌＤ，PennockJ，Ｇ()()〔ｌｗｉｎＦ，ｅｔａｌ：
Ｓｅｑｕｅｎｔｉａｌｓｔｕｄｙｏｆｃｅｎｔｒａｌａｎｄｌ〕eripheralnervoussystominvolvｅｍｃｎｔｉｎａｎｉｎ－
ｆａｎｔｗｉｔｈｍｅｒｏｓｉｎ－deficientcongeniLalmusculLu・dysLrophy．Ｎｅｕｒ()muscular24）Ｙ〔〕shiokaM，（)kunoT，IIondaY，ｅｔａｌ：
Ccntralnervoussysteminvolvemenｔｉｎｌ〕rogressivemusculardysLrophy、Arch
DisChildl980；５５：589-594.

25）ＯｚａｗａＥ，YoshidaM，SuzukiA，ｅｔａｌ：

Dystrophin-associatedglycoprotｅｉｎｓｉｎ
ｍｕｓｃｕｌａｒ〔lystl･()phy，ＩＩｕｍＭｏｌＧｃｎｅｔ
ｌ９９５；４：１７１１－１７１６．

２６）Ｌｏｉｖｏｌ，ILngvallE：Mor()sin，aprotein
specific「oｒｂａｓｅｍｃｎｔｍｃｍｂｒａｎｅｓｏ（

Ｓｃｈｗａｎｎｃｏｌｌｓ，striaLedmuscle，ａｎｄｔｒｏ‐
ｐｈｏｂｌａｓｔ，ｉｓｅｘｌ)ｒｏｓｓｅｄｌａＬｅｉｌｌｎｅｒｖｅａｎｄｍｕｓｃｌｅｄｅvelopmenLI〕r()ｃＮａｔｌＡｃａｄＳｃｉ
ＵＳＡ１９８８；８５：1544-1548.

27）Ｌｅｉｖｏｌ，Laurilal〕，WahlstromT，ｅｔａｌ：
Expressionofmerosill，aLissue-specificbasementmcml)raneprotcin，ｉｎｔｈｅｉｎ－ｔｅｒｍｅｄｉａｔｏｔｒ()ｐｈｏｂｌａｓtcellsofchoriocar‐

ｃｉｎｏｍａａｎｄｐｌａｃｅｎｔａ、LaboraLoryinvesti‐

gationl989；６０：783-790.

28）ＣｈａｎｇＡＣ，Ｗｌｌ(lsworthS，ColiganJE：
Exprossi〔)ｎｏＩｍｅｒｏｓｉｎｉｎｔｈｃｔｈｙｍｕｓａｎｄ
ｉｔｓｉｎteractionwiLhLhymocytes・ＪＩｍ‐
ｍｕｎｏｌｏｇｙｌ９９３；１５１：1789-1801.

29）LoivoLEngvallID，Laurilal〕，ｅｔａｌ：Dis‐
ｔｒｉｂｕｔｉｏｎｏｌ．mer()sｉｎ，ａｌａｍｉｎｉｎ－Ｉ､elaLed
Lissue-spocificbasementmcmbraneprotein，
ｉｎｈｕｍａｎｓｃｈｗａｎｎｃｏｌｌｎｅｏｐｌａｓｍｓ・Ｌａｂ()‐
ratorylnvesljgalj()、1989；６１：`126-432.
30）HavashiYK，IlngvaJllD，ＡｒｉｋａｗａＨＥ，ｅｔａｌ：Ａｂｎｏｒｍａｌｌｏｃａｌｉｚａｔｉｏｎｏｆｌａｍｉｎｉｎ

ｓｕｂｕｎｉｔｓｉｎｍｕｓｃｕｌａｒｄｙｓｔｒｏｐｈｉｅｓ・ＪＸｅｕ‐
ｒｏｌＳｃｉｌ９９３；１１９：53-64.

31）McrcuriE，JIuI1LoniIJ､，BcrardinelliA，ｅｔａｌ：Somatosonsoryandvisualevokedpo‐
ｔｅｎｔｉａｌｓｉｎｃｏｎｇｅｎｉｔａｌｍｕｓｃｕｌａｒｄｙstro‐
ｐｈｙ：ｃｏｒｒｅｌａｔｉｏｎｗｉｔｈＭＲＩｃｈａｎｇｅｓａｎｄｍｕsclemerosinstatus、Ncuropediatrics
l995；２６：３－７．

Disor[Iersl996；(沖125-429.

5９