1333 Müller cell abnormalities in Duchenne muscular dystrophy

نویسندگان

  • M. Warburg
  • O. Sjö
  • H. Jensen
  • M. Schwartz
  • S. E. Simonsen
چکیده

INNER-RETINAL COMPONENTS CONTRIBUTE TO THE A-WAVE OF THE ERG OF THE DARK-ADAPTED CAT. Robson J.G.1 and Frishman L.J.2 ’ Physiological Laboratory, Cambridge CE2 3EG UK/ 2 College of Optomern/ UniversitY of Houston, Houston. TX 77204 fUSA/ Purpose To determine the extent to which the initial negative wave and its return to baseline (the a-wave) of the flash electroretinogram IERG) can be modelled simply as the sum of a negative photoreceptor component and a positive bipolar-cell component. Method We have examined the time-course of the vitreally recorded a-wave in the urethane-anaesthetised cat and the effect upon it of suppressing the responses of inner-retinal Iamacrine or ganglion) cells either with intra-vitreal injection of n-methyl-oL-aspartate [NMoLA) or by application of a steady adapting background of moderate intensity known to affect mainly responses of inner retina. Resuhs The time-course of the a-wave at times more than about 10 msec after the flash is greatly altered by both NMDLA and adapting backgrounds. The most obvious effect of both procedures, removal of a negative component, occurred not only when the stimulus was weak enough for the negative STR to dominate under normal condidons, but also when the stimulus was strong enough for the rods themselves to contribute directly to the a-wave. However, once the responses of inner-retinal cells had been suppressed, the remaining a-wave could be satisfactorily explained as the sum of photoreceptor and bipolar-cell responses modelled as described by Lamb & Pugh I./. Physiol. 449, ~719, 1992) and Robson & Frishman (Vis. Neurosci. 12, 1995) respectively. Conclusion Inner-retinal components contribute substantially to the a-wave of the dark-adapted ERG. Only when these responses have been blocked pharmacologically, or have been sufficiently reduced by an adapting background, can the initial negative wave of the ERG be simply explained as the sum of photoreceptor and bipolar-cell components. Supwrted by EY0667 1.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

P164: Adeno-Associated Viral Vectors in Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (BMD) is an inherited X-link disease. The incidence of this muscle-wasting disease is 1:5000 male live births. Mutation in the gene coding for dystrophin is the main cause of BMD. Most cases of this disease succumb to respiratory and cardiac failure in 3rd to 4th decades. The slow progression of BMD and recent achievement of gene therapies make it as an appropriate c...

متن کامل

Detection of the Duplication in Exons 56-63 of Duchenne Muscular Dystrophy Patients with MLPA

Background Duchenne Muscular Dystrophy (DMD) is a deadly X-linked recessive disorder. This genetic disorder affects 1 among 3,500-5,000 males in the world. The majority of the patients are male, due to the type of inheritance. It affects most of the skeletal, the respiratory, and cardiac muscles, causing these vital organs to contract and eventually mortality.<br...

متن کامل

Theoretical considerations on germline mosaicism in Duchenne muscular dystrophy.

A newly formulated mutation selection equilibrium for lethal X linked recessive traits such as Duchenne muscular dystrophy is presented, which allows for both male and female germline mosaicism. Estimates of the additional parameters used are given, thus allowing the incorporation of germline mosaicism into the calculation of genetic risks.

متن کامل

Satellite Cells: Regenerative Mechanisms and Applicability in Muscular Dystrophy

The satellite cells are long regarded as heterogeneous cell population, which is intimately linked to the processes of muscular recovery. The heterogeneous cell population may be classified by specific markers. In spite of the significant amount of variation amongst the satellite cell populations, it seems that their activity is tightly bound to the paired box 7 transcription factor expression,...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • Vision Research

دوره 35  شماره 

صفحات  -

تاریخ انتشار 1995