A Review of Mathematical Models for Muscular Dystrophy: A Systems Biology Approach

نویسندگان

  • Amanda N. Cameron
  • Matthew T. Houston
  • Juan B. Gutierrez
چکیده

Muscular dystrophy (MD) describes generalized progressive muscular weakness due to the wasting of muscle fibers. While this is an umbrella term used to describe a wide range of muscle wasting diseases, the two types that have been most extensively mathematically modeled are Duchenne’s (DMD) and Becker’s (BMD), with DMD being the most common childhood form of MD. Developing mathematical models to understand and treat MD involves the integration of previously established immunological and mechanical representations of these factors that bolster disease progression. This article reviews these mathematical models and examines key molecular and cellular perspectives to better understand MD’s pathogenesis and subsequent progression. Molecular factors that contribute to MD include mitochondrial and genetic components; both drive cellular metabolism, communication and signaling. Furthermore, these molecular factors leave cells vulnerable to mechanical stress which can activate an immunological cascade that can weaken cells and surrounding tissues. This review article provides an essential, brief, biological background for mechanisms modeled. Given the application of developed immunological and molecular models to a wider variety of neurodegenerative diseases linked by related mitochondrial and genetic deficits, understanding and modeling MD is highly applicable.

برای دانلود 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

Association of Limb-Girdle muscular dystrophy with multiple sclerosis: A case report

Background: The association of limb-girdle muscular dystrophy (LGMD) with other neurological disorders is uncommon. Case presentation: We report a 25-year-old female with LGMD who suffered from slowly progressive proximal muscular weakness and atrophy since she was 12 years of age. The patient recently presented with acute loss of left side visual acuity. After evaluation, findings were sugges...

متن کامل

بیحسی داخل نخاعی با بوپیواکایین جهت جراحی سزارین اورژانسی

Aim and Background: Becker’s muscular dystrophy (BMD) is similar to the Duchenne’s muscular dystrophy, but the clinical course is milder.We introduced a patient with Becker Muscle Dystrophy who candidate for emergency cesarean section.Case report:A36-year-old woman, gestational age 31 weeks, with a history of Becker Muscle Dystrophy and fetal distress who candidate for emergency cesarean sectio...

متن کامل

The Diagnostic Value of Utrophin in Mild Dystrophinopathy (Becker Muscular Dystrophy)

  Background and Objective: Becker Muscular Dystrophy (BMD) is a subtype of dystrophinopathies and designated as “mild form of dystrophinopathy”. The frequency rate of the disease is 1:18000 to 1:30000 in different populations and the symptoms are presented at about 8-9 years of age. The diagnostic panel composed of Serum Ceratin Kinase (SCK) measurement, Electromyography (EMG), and as a major...

متن کامل

A pathogenic variant in the transforming growth factor beta I (TGFBI) in four Iranian extended families segregating granular corneal dystrophy type II: A literature review

Objective(s): Granular and lattice corneal dystrophies (GCDs & LCDs) are autosomal dominant inherited disorders of the cornea. Due to genetic heterogeneity and large genes, unraveling the mutation is challenging.Materials and Methods: Patients underwent comprehensive clinical examination, and targeted next-generation sequencing (NGS) was...

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

برای دانلود 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

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2016