Applicability of 19–DEJ‐l monoclonal antibody for the prenatal diagnosis or exclusion of junctional epidermolysis bullosa
Applicability of 19–DEJ‐l monoclonal antibody for the prenatal diagnosis or exclusion of junctional epidermolysis bullosa
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19-DEJ-1单克隆抗体在产前诊断或排除大疱性交界性表皮松解症中的应用
作者：
J. Fine;K. Holbrook;S. Elias;I. Anton‐Lamprecht;R. Rauskolb
最近产生了一种单克隆抗体（19-DEJ - 1），具有结合特异性，用于皮肤真皮表皮交界处的中透明层，与上覆半脂体密切相关。由于遗传性水泡性疾病大疱性结缔组织松解症（EB）的皮肤分裂发生在透明层内，并且与该组织中半粒粒的形态和/或数量的畸变有关，我们试图确定这种单克隆抗体是否可用于产前诊断。胎儿镜指导下的皮肤活检来自两个有结缔性EB风险的胎儿，以及另外两个患有Herlitz型结缔性EB的胎儿的尸检样本，后者在产前通过电子显微镜诊断并终止妊娠后。标本部分通过光学和电子显微镜检查皮肤劈裂或其他形态学改变的证据，部分通过间接免疫荧光检查基底膜抗原性的改变。4个胎儿中有3个显示出板内透明水疱的形成，表明有半脂小体发育不全，证明有交界性EB。每一个都显示缺乏GB3和19-DEJ - 1抗原的表达，这与出生后在结缔组织EB中发现的结果一致；在治疗性流产时确诊。第四个胎儿没有发现异常；在分娩时确认无疾病累及，随后进行了8个多月的仔细系列评估。我们认为，19-DEJ - 1单克隆抗体是一种准确、敏感的连接型EB免疫组织化学探针，可用于产前诊断评估这种疾病的风险胎儿。
Recently a monoclonal antibody (19–DEJ‐l) was produced with binding specificity for the mid‐lamina lucida of the skin dermoepidermal junction, in very close association with overlying hemidesmosomes. Since skin cleavage occurs within the lamina lucida in the inherited blistering disorder, junctional epidermolysis bullosa (EB), and is associated with aberrations in the morphology and/or number of hemidesmosomes in such tissue, we have sought to determine whether this monoclonal antibody could be used for prenatal diagnosis. Fetoscopy‐directed skin biopsies were obtained from two fetuses at risk for junctional EB and post‐mortem samples from two other fetuses with the Herlitz type of junctional EB, the latter after prenatal diagnosis by electron microscopy and termination of each pregnancy. Specimens were examined in part by light and electron microscopy for evidence of skin cleavage or other alterations in morphology, and in part by indirect immunofluorescence for altered basement membrane antigenicity. Three of four fetuses were shown to have intra‐lamina lucida blister formation indicative of, and hemidesmosome hypoplasia proving, junctional EB. Each was also shown to lack expression of GB3 and 19–DEJ‐l antigens, consistent with findings noted postnatally in junctional EB; diagnosis was confirmed in each at the time of therapeutic abortion. A fourth fetus had no abnormalities detected; lack of disease involvement was confirmed at the time of delivery, and subsequently over 8 months of careful serial evaluation. We conclude that 19–DEJ‐l monoclonal antibody is an accurate and sensitive irnmunohistochemical probe for junctional EB, and may be employed in the prenatal diagnostic evaluation of fetuses at risk for this disorder.
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显性和隐性营养不良性大疱性表皮松解症的产前诊断：KF-1和LH 7:2单克隆抗体和免疫荧光图谱技术的应用和局限性。
DOI：
10.1111/1523-1747.ep12476604
发表时间：
1988
期刊：
The Journal of investigative dermatology
影响因子：
--
作者：
Fine,JD;Eady,RA;Levy,ML;Hejtmancik,JF;Courtney,KB;Carpenter,RJ;Holbrook,KA;Hawkins,HK
通讯作者：
Hawkins,HK
19-DEJ-1，一种半桥粒锚定丝复合物相关的单克隆抗体。
影响因子：
--
作者：
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通讯作者：
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VII 型胶原蛋白的羧基末端结构域存在于隐性营养不良性大疱性表皮松解症的基底膜上。
DOI：
10.1111/1523-1747.ep12712167
发表时间：
1989
期刊：
The Journal of investigative dermatology
影响因子：
--
作者：
Rusenko,KW;Gammon,WR;Fine,JD;Briggaman,RA
通讯作者：
Briggaman,RA
19-DEJ-1 是半桥粒锚定丝复合物的单克隆抗体，是用于所有主要形式的交界性大疱性表皮松解症的唯一可靠的免疫组织化学探针。
影响因子：
--
作者：
Fine,JD
通讯作者：
Fine,JD
锚定原纤维的单克隆抗体用于诊断大疱性表皮松解症。
DOI：
10.1111/1523-1747.ep12522669
发表时间：
1983
期刊：
The Journal of investigative dermatology
影响因子：
--
作者：
Goldsmith,LA;Briggaman,RA
通讯作者：
Briggaman,RA