Breaking News
March 23, 2019 - People with peripheral artery disease have lower Omega-3 Index, shows research
March 23, 2019 - Trigger warnings have minimal impact on how people respond to content, shows research
March 23, 2019 - Gilead Announces Data From Two Studies Supporting Further Development of GS-6207, a Novel, Investigational HIV-1 Capsid Inhibitor as a Component of Future Long-Acting HIV Therapies
March 23, 2019 - Selfish genetic elements amplify inflammation and age-related diseases
March 23, 2019 - Study provides new understanding of how the brain recovers from damage caused by stroke
March 23, 2019 - CRISPR/Cas libraries could revolutionize drug discovery
March 23, 2019 - Allergic reaction during pregnancy may alter sexual-development in offspring’s brain
March 23, 2019 - Seeing through a robot’s eyes helps those with profound motor impairments
March 23, 2019 - Recent research shows that ease of breastfeeding after C-section differs culturally
March 23, 2019 - Newly discovered parameters offer more control over efficient release of drugs
March 23, 2019 - ‘De-tabooing’ of abortion- Women would like more support from health care community
March 23, 2019 - Anti-TB drugs can increase susceptibility to Mtb reinfection
March 23, 2019 - New survey indicates need of attention to neglected tropical diseases
March 23, 2019 - Innovative in vitro method to develop easy-to-swallow medicine for children and older people
March 23, 2019 - Sugary drinks could raise risk of early deaths finds study
March 23, 2019 - Lian wins ENGINE grant for stem-cell-based therapy to treat Type 1 diabetes
March 23, 2019 - Overall, Physicians Are Happy and Enjoy Their Lives
March 23, 2019 - Researchers discover how blood vessels protect the brain during inflammation
March 23, 2019 - CDC study shows modest improvement in optimal hospital breastfeeding policy
March 23, 2019 - Family-based prevention program to reduce alcohol use among older teens
March 23, 2019 - Remote monitoring of implanted defibrillators in heart failure patients prevents hospitalizations
March 23, 2019 - Appropriate doffing of personal protective equipment may reduce healthcare worker contamination
March 23, 2019 - Window screens can suppress mosquito populations, reduce malaria in Tanzania
March 23, 2019 - Researchers discover new biomarker for postoperative liver dysfunction
March 23, 2019 - Pregnancy history may be linked to cognitive function in older women, finds study
March 23, 2019 - Study shows ticagrelor is equally safe and effective as clopidogrel after heart attack
March 23, 2019 - FDA Approves First Drug for Postpartum Depression, Zulresso (brexanolone)
March 23, 2019 - New guidelines outline new treatment management for psoriasis
March 23, 2019 - Thermally abused cooking oil may promote progression of breast cancer
March 23, 2019 - High-fructose corn syrup fuels growth of colon tumors in mice
March 23, 2019 - Partnership aims at establishing best practices to promote diversity in clinical trials
March 23, 2019 - New study examines presence of microbes in tap water from residences, office buildings
March 23, 2019 - Early life trauma may affect brain structure, contribute to major depressive disorder
March 23, 2019 - NIH starts clinical trial of drug to treat cravings associated with opioid use disorder
March 23, 2019 - Cervix bacteria, immune factors could be a warning signal of premature birth, reports new research
March 23, 2019 - Worst-ever emergency care performance figures underscore the need to focus on staffing
March 23, 2019 - The Current issue of “The view from here” is concerned with Cancer
March 23, 2019 - Mouse model validates how ‘good’ and ‘bad’ bacteria affect acne
March 23, 2019 - Individual amygdala neurons respond to touch, imagery and sounds
March 23, 2019 - Combination of two topical creams can prevent cancer
March 23, 2019 - Study suggests depression screening when assessing African-Americans for schizophrenia
March 23, 2019 - New electronic support system for choosing drug treatment based on patient’s genotype
March 23, 2019 - First-of-its-kind study provides pregnancy statistics of imprisoned U.S. women
March 23, 2019 - Marinus Pharmaceuticals Initiates Phase 3 Study in Children with PCDH19-Related Epilepsy
March 23, 2019 - Laparoscopy: MedlinePlus Lab Test Information
March 23, 2019 - Shellfish allergies: can they be treated?
March 23, 2019 - Toilet seat heart monitoring system
March 23, 2019 - Researchers identify way to improve common treatment for PTSD
March 23, 2019 - High potency cannabis use linked to psychosis finds study
March 23, 2019 - Evoke Pharma Submits Response to FDA Review Letter for Gimoti NDA
March 23, 2019 - Tracking HIV’s ever-evolving genome in effort to prioritize public health resources
March 23, 2019 - Scientists grow most sophisticated brain organoid to date
March 23, 2019 - ADHD drug raising risk of psychosis
March 22, 2019 - FDA approves brexanolone, first drug developed to treat postpartum depression
March 22, 2019 - Gruesome cat and dog experiments by the USDA exposed
March 22, 2019 - Ball pits used in children’s physical therapy may contribute to germ transmission
March 22, 2019 - Long-term use of inexpensive weight-loss drug may be safe and effective
March 22, 2019 - FDA Approves Sunosi (solriamfetol) for Excessive Daytime Sleepiness Associated with Narcolepsy or Obstructive Sleep Apnea
March 22, 2019 - Anti-Müllerian Hormone Test: MedlinePlus Lab Test Information
March 22, 2019 - Finding the right exercise, diet aids for HIV patients
March 22, 2019 - Health Plans For State Employees Use Medicare’s Hammer On Hospital Bills
March 22, 2019 - Researchers develop new tool for imaging large groups of neurons in living animals
March 22, 2019 - Certain bacteria and immune factors in vagina may cause or protect against preterm birth
March 22, 2019 - Research identifies guidelines for prioritizing hepatitis C treatment in U.S. prisons
March 22, 2019 - Novel breath test could pave new way to non-invasively measure gut health
March 22, 2019 - Pharmaceutical and personal care products may result in new contaminants in waterways
March 22, 2019 - New model could revolutionize the way researchers investigate spread of pathogens
March 22, 2019 - MSU professor receives NSF CAREER grant for biosensor diagnostics
March 22, 2019 - High-fat, high-sugar diet in mouse mothers causes problems in the hearts of offspring
March 22, 2019 - ACC: Catheter Ablation Does Not Cut Mortality, Stroke in A-Fib
March 22, 2019 - Wiedemann-Rautenstrauch syndrome – Genetics Home Reference
March 22, 2019 - Health insurance is not assurance of healthcare
March 22, 2019 - Supporting “curiosity-driven research” at the Discovery Innovation Awards
March 22, 2019 - Must-Reads Of The Week (Some Flying Below The Radar)
March 22, 2019 - Newly engineered nanoscale protein micelles can be tracked by MRI
March 22, 2019 - New model more effective in predicting risk of opioid overdose than traditional models
March 22, 2019 - Mayo Clinic study identifies potential new drug therapy for liver diseases
March 22, 2019 - Pitt engineers win $550,000 NSF CAREER award to develop new intervention for people with ASD
March 22, 2019 - Early discharge does not increase readmission risk for patients after lung surgery
March 22, 2019 - Creating diverse pool of trained scientists to address Alzheimer’s research needs
How a single mutation can devastate cellular function

How a single mutation can devastate cellular function

image_pdfDownload PDFimage_print

A professor of neuroscience at the University of Wisconsin-Madison has shown how a single mutation in a protein found in astrocytes reproduces fibrous globs that devastate cellular function.

By Anusorn NakdeeImage Credit: Anusorn Nakdee / Shutterstock

The globs disrupt the positioning of cellular processing units, interrupt signal and energy within the brain and hinder the formation of the nerve insulator myelin.

A pioneer in neural cell studies, Professor Su-Chun Zhang, MD, Ph.D. looked at astrocytes, which play many roles in the brain including supplying nutrients, supporting cells in the blood-brain barrier, regulating calcium ion balance and aiding insulation of nerves with myelin.

For the study, Zhang took adult cells from the families of two patients with the rare and fatal genetic condition Alexander disease and converted them into stem cells, from which he could grow astrocytes.

The astrocytes he grew displayed the hallmarks of Alexander disease, including tangles made up of a protein called glial fibrillary acidic protein (GFAP) and mitochondria that were misplaced along with other cellular processing units.

As reported in the journal Cell Reports, when Zhang used CRISPR-Cas9 gene editing to correct the stem cells, the astrocytes no longer displayed any signs of Alexander disease.

Alexander disease is itself an extremely rare condition, says Zhang, but rare diseases are important in neuroscience:

We often grow to understand a disease process through rare diseases. One mutation discovered in a family with Lou Gehrig’s disease led to the discovery of the fundamental pathogenesis of ALS, and the same is true for Parkinson’s and Alzheimer’s.”

Professor Su-Chun Zhang, Lead Author

The damage caused by GFAP seems to begin with filaments aggregating within the astrocytes and causing widespread tangles that probably trigger disruption of the organelles involved in protein production, energy processing and chemical storage:

The mitochondria, endoplasmic reticulum, and lysosomes were all distributed abnormally and that was a clue that GFAP is critical for guiding organelles to their correct locations.”

Professor Su-Chun Zhang, Lead Author

Although the findings do not have any immediate clinical implications, Zhang says the impact could be broad since GFAP is the most abundant protein in astrocytes and is altered in nearly all neurological conditions including Alzheimer’s, Parkinson’s, Huntington’s, ALS and autism.

Source:

https://www.eurekalert.org/emb_releases/2018-10/uow-mic101918.php

Tagged with:

About author

Related Articles