中华眼底病杂志
中華眼底病雜誌
중화안저병잡지
CHINESE JOURNAL OF OCULAR FUNDUS DISEASES
2014年
3期
289-293
,共5页
韩娟娟%戴旭锋%张华%戚艳%庞继景
韓娟娟%戴旭鋒%張華%慼豔%龐繼景
한연연%대욱봉%장화%척염%방계경
卵磷脂酰基转移酶缺乏症%视网膜变性/病理学%视网膜电描记术
卵燐脂酰基轉移酶缺乏癥%視網膜變性/病理學%視網膜電描記術
란린지선기전이매결핍증%시망막변성/병이학%시망막전묘기술
Lecithin acyltransferase deficiency%Retinal degeneration/pathology%Electroretinography
目的 观察溶血卵磷脂酰基转移酶1(LPCAT1)自发突变小鼠视网膜组织和闪光视网膜电图(F-ERG)改变情况.方法 Lpcat1基因自发突变纯合子的rd11新生小鼠60只(实验组)和与之同龄的野生型C57BL/6J小鼠60只(对照组)纳入实验.采用免疫荧光法检测两组小鼠视网膜组织中LPCAT1的表达情况.出生后3、6、9d和2、4、6、8周,行视网膜石蜡切片观察视网膜显微结构;行全视野F-ERG检测,记录暗视杆体反应和明视锥体反应.结果 实验组小鼠视网膜组织中LPCAT1呈阴性表达;对照组小鼠视网膜组织中LPCAT1呈阳性表达,主要分布于视网膜光感受器内节,神经节细胞层可见少量表达.光学显微镜观察发现,实验组小鼠于出生后9d左右视网膜外核层已基本形成,其光感受器细胞核层数随年龄增长而逐渐减少.对照组小鼠视网膜显微结构正常.F-ERG检测结果显示,实验组小鼠出生后2、4周暗视杆体系统反应存在,呈降低趋势,出生后6～8周暗视杆体系统反应消失;对照组小鼠各时间点暗视杆体系统反应正常.出生后2周,实验组和对照组小鼠b波振幅分别为(72.8±15.6)、(105.2±21.1) μV,实验组较对照组降低,但差异无统计学意义(t=-2.760,P=0.025).出生后4周,实验组和对照组小鼠b波振幅分别为(20.6±6.4)、(231.8±32.0)μV,实验组较对照组明显降低,差异有统计学意义(t=-14.471,P=0.000).实验组小鼠出生后2、4、6周明视锥体系统反应存在,呈降低趋势,出生后8周明视锥体系统反应消失;对照组小鼠各时间点明视锥体系统反应正常.出生后2、4周,实验组小鼠b波振幅分别为(46.8±7.2)、(78.0±8.2)μV;对照组小鼠b波振幅分别为(42.8±6.4)、(91.4±9.4) μV.两组比较,差异均无统计学意义(t=0.930、-2.401,P=0.379、0.043).出生后6周,实验组和对照组小鼠b波振幅分别为(17.2±2.0)、(116.2±12.9) μV,实验组较对照组明显降低,差异有统计学意义(t=-17.008,P=0.000).结论 LPCAT1自发突变小鼠视网膜外层光感受器细胞核层数随年龄增长而逐渐减少,F-ERG暗视杆体和明视锥体反应异常.
目的 觀察溶血卵燐脂酰基轉移酶1(LPCAT1)自髮突變小鼠視網膜組織和閃光視網膜電圖(F-ERG)改變情況.方法 Lpcat1基因自髮突變純閤子的rd11新生小鼠60隻(實驗組)和與之同齡的野生型C57BL/6J小鼠60隻(對照組)納入實驗.採用免疫熒光法檢測兩組小鼠視網膜組織中LPCAT1的錶達情況.齣生後3、6、9d和2、4、6、8週,行視網膜石蠟切片觀察視網膜顯微結構;行全視野F-ERG檢測,記錄暗視桿體反應和明視錐體反應.結果 實驗組小鼠視網膜組織中LPCAT1呈陰性錶達;對照組小鼠視網膜組織中LPCAT1呈暘性錶達,主要分佈于視網膜光感受器內節,神經節細胞層可見少量錶達.光學顯微鏡觀察髮現,實驗組小鼠于齣生後9d左右視網膜外覈層已基本形成,其光感受器細胞覈層數隨年齡增長而逐漸減少.對照組小鼠視網膜顯微結構正常.F-ERG檢測結果顯示,實驗組小鼠齣生後2、4週暗視桿體繫統反應存在,呈降低趨勢,齣生後6～8週暗視桿體繫統反應消失;對照組小鼠各時間點暗視桿體繫統反應正常.齣生後2週,實驗組和對照組小鼠b波振幅分彆為(72.8±15.6)、(105.2±21.1) μV,實驗組較對照組降低,但差異無統計學意義(t=-2.760,P=0.025).齣生後4週,實驗組和對照組小鼠b波振幅分彆為(20.6±6.4)、(231.8±32.0)μV,實驗組較對照組明顯降低,差異有統計學意義(t=-14.471,P=0.000).實驗組小鼠齣生後2、4、6週明視錐體繫統反應存在,呈降低趨勢,齣生後8週明視錐體繫統反應消失;對照組小鼠各時間點明視錐體繫統反應正常.齣生後2、4週,實驗組小鼠b波振幅分彆為(46.8±7.2)、(78.0±8.2)μV;對照組小鼠b波振幅分彆為(42.8±6.4)、(91.4±9.4) μV.兩組比較,差異均無統計學意義(t=0.930、-2.401,P=0.379、0.043).齣生後6週,實驗組和對照組小鼠b波振幅分彆為(17.2±2.0)、(116.2±12.9) μV,實驗組較對照組明顯降低,差異有統計學意義(t=-17.008,P=0.000).結論 LPCAT1自髮突變小鼠視網膜外層光感受器細胞覈層數隨年齡增長而逐漸減少,F-ERG暗視桿體和明視錐體反應異常.
목적 관찰용혈란린지선기전이매1(LPCAT1)자발돌변소서시망막조직화섬광시망막전도(F-ERG)개변정황.방법 Lpcat1기인자발돌변순합자적rd11신생소서60지(실험조)화여지동령적야생형C57BL/6J소서60지(대조조)납입실험.채용면역형광법검측량조소서시망막조직중LPCAT1적표체정황.출생후3、6、9d화2、4、6、8주,행시망막석사절편관찰시망막현미결구;행전시야F-ERG검측,기록암시간체반응화명시추체반응.결과 실험조소서시망막조직중LPCAT1정음성표체;대조조소서시망막조직중LPCAT1정양성표체,주요분포우시망막광감수기내절,신경절세포층가견소량표체.광학현미경관찰발현,실험조소서우출생후9d좌우시망막외핵층이기본형성,기광감수기세포핵층수수년령증장이축점감소.대조조소서시망막현미결구정상.F-ERG검측결과현시,실험조소서출생후2、4주암시간체계통반응존재,정강저추세,출생후6～8주암시간체계통반응소실;대조조소서각시간점암시간체계통반응정상.출생후2주,실험조화대조조소서b파진폭분별위(72.8±15.6)、(105.2±21.1) μV,실험조교대조조강저,단차이무통계학의의(t=-2.760,P=0.025).출생후4주,실험조화대조조소서b파진폭분별위(20.6±6.4)、(231.8±32.0)μV,실험조교대조조명현강저,차이유통계학의의(t=-14.471,P=0.000).실험조소서출생후2、4、6주명시추체계통반응존재,정강저추세,출생후8주명시추체계통반응소실;대조조소서각시간점명시추체계통반응정상.출생후2、4주,실험조소서b파진폭분별위(46.8±7.2)、(78.0±8.2)μV;대조조소서b파진폭분별위(42.8±6.4)、(91.4±9.4) μV.량조비교,차이균무통계학의의(t=0.930、-2.401,P=0.379、0.043).출생후6주,실험조화대조조소서b파진폭분별위(17.2±2.0)、(116.2±12.9) μV,실험조교대조조명현강저,차이유통계학의의(t=-17.008,P=0.000).결론 LPCAT1자발돌변소서시망막외층광감수기세포핵층수수년령증장이축점감소,F-ERG암시간체화명시추체반응이상.
Objective To observe the effect of lysophosphatidylcholine acyltransferase-1 (LPCAT1)deficiency on the structure and electrophysiology of the murine retina.Methods Rd11 mice (Lpcat1 homozygous mutant,n =60) and wild-type C57BL/6J mice (n =60) were used in this study.Immunohistochemistry was performed to determine the expression of LPCAT1 in the mouse retina.Retinas of rd11 mice and age-matched control mice at postnatal 3,6,9-day and 2,4,6,8-week were paraffin embedded,sectioned and hematoxylin eosin stained,and full-field electroretinograms (F-ERG) were also recorded at these time points.Statistics were based on independent samples t-test.Results LPCAT1 was absent in rd11 mice retina.In wild-type C57BL/6J mice retina,LPCAT1 was expressed most strongly in the inner segment of photoreceptor cells,weak in the ganglion cell layer.Rd11 mouse exhibited retinal degeneration and eventual photoreceptor cell loss.Retinas of rd11 mice showed nearly half of the photoreceptor cells missing around postnatal week 4,and by 6-week after birth only two layers of nuclei remained.At postnatal week 8,nearly all photoreceptor cells were lost.Dark-adapted F-ERG showed reduced rod-driven response at 2 and 4 weeks of age,which was flattened by 6 and 8 weeks of age.By 2 weeks of age,no significant difference was found in b-wave amplitude between rd11 eyes [(72.8±15.6) μV]and C57BL/6J eyes [(105.2±21.1) μV] (t=-2.760,P=0.025).Compared with age-matched control mice [(231.8±32.0)μV],rod response of rd11 mice [(20.6±6.4) μV] decreased obviously at postnatal week4 (t=-14.471,P=0.000).Cone response was nearly normal at 2 and 4 weeks of age but substantially reduced at 6 weeks of age,which was flattened by 8 weeks of age.At 2 and 4 weeks after birth,no significant difference was found in b-wave amplitude between rd11 eyes [(46.8 ± 7.2),(78.0 ±8.2) μV] and C57BL/6J eyes [(42.8±6.4),(91.4±9.4) μV] (t=0.930,-2.401; P=0.379,0.043).Compared with age-matched control mice [(116.2 ± 12.9) μV],cone response of rd11 mice [(17.2 ± 2.0) μV]decreased obviously at postnatal week 6 (t =-17.008,P =0.000).Conclusion The layers of photoreceptor cells nuclei in rd11 mice decreases with age,and its F-ERG reflection is unusual.
