Navigation Links
Researchers untangle molecular pathology of giant axonal neuropathy
Date:4/16/2013

Giant axonal neuropathy (GAN) is a rare genetic disorder that causes central and peripheral nervous system dysfunction. GAN is known to be caused by mutations in the gigaxonin gene and is characterized by tangling and aggregation of neural projections, but the mechanistic link between the genetic mutation and the effects on neurons is unclear. In this issue of the Journal of Clinical Investigation, Robert Goldman and colleagues at Northwestern University uncover how mutations in gigaxonin contribute to neural aggregation.They demonstrated that gigaxonin regulates the degradation of neurofilament proteins, which help to guide outgrowth and morphology of neural projections. Loss of gigaxonin in either GAN patient cells or transgenic mice increased levels of neurofilament proteins, causing tangling and aggregation of neural projections. Importantly, expression of gigaxonin allowed for clearance of neurofilament proteins in neurons. These findings demonstrate that mutations in gigaxonin cause accumulation of neurofilament proteins and shed light on the molecular pathology of GAN.


'/>"/>

Contact: Jillian Hurst
press_releases@the-jci.org
Journal of Clinical Investigation
Source:Eurekalert

Page: 1

Related biology technology :

1. Top BioEnergy Researchers and Innovators Arriving Next Week to Foster U.S.-Israel Collaboration
2. Researchers identify transcription factors that regulate retinal vascularization
3. Bean leaves can trap bedbugs, researchers find
4. Elsevier and the Belarus Ministry of Education Honor Researchers with Scopus Awards
5. Beekeepers, Researchers and Bayer CropScience Associate Overwintering Bee Losses with Varroa Mite
6. Researchers build functional ovarian tissue in lab
7. RI Hospital researchers discover new strategy to effectively treat, prevent osteoarthritis
8. Ingenuity Systems IPA Provides Researchers with Genomic Insights Suggesting that Physical Activity Does Not Slow Muscle Aging
9. Berkeley Lab researchers use metamaterials to observe giant photonic spin hall effect
10. Leading Photonics and Laser Researchers to Present Latest Work at SPIE Optics + Optoelectronics
11. The Hartwell Foundation Provides Grant to University of Michigan Researchers
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:12/19/2014)... 18, 2014   ISN has released its ... Indicators publication series. With data aggregated from ... in 2013 within its online contractor management platform, ISNetworld, ... performance indicator (KPI) statistics. The ... publication is available in two industry specific volumes: ...
(Date:12/17/2014)... NY (PRWEB) December 16, 2014 The ... changes since 2013, which is why IBISWorld updated its ... industry continues to benefit from an intensified focus on ... proposal for new emission standards for power plants and ... operators. According to IBISWorld Industry Analyst Sarah Kahn, “a ...
(Date:12/17/2014)... “Never say, ‘I cannot do ... Department of Physics, advises young women pursuing careers ... SPIE Women in Optics 18-month planner ... ask questions.” , In the annual planner, women ... ranging from university professor and laboratory researcher to ...
(Date:12/17/2014)... 17, 2014 Ipsen Biopharmaceuticals, Inc., ... ADR: IPSEY), today announced that Somatuline® ... Somatuline®) was approved by the U.S. Food and ... neuroendocrine tumors (GEP-NETs) in adult patients with unresectable, ... disease to improve progression-free survival (PFS). , “The ...
Breaking Biology Technology:ISN Releases Third Edition of US Contractor Health & Safety Performance Indicators Publication Series 2ISN Releases Third Edition of US Contractor Health & Safety Performance Indicators Publication Series 3Activated Carbon Manufacturing in the US Industry Market Research Report Has Been Updated from IBISWorld 2Activated Carbon Manufacturing in the US Industry Market Research Report Has Been Updated from IBISWorld 3Network, Multitask, Persevere: SPIE Women in Optics Planner Subjects Share from Experience 2Network, Multitask, Persevere: SPIE Women in Optics Planner Subjects Share from Experience 3Ipsen Announces the FDA Approval of Somatuline® Depot® (lanreotide) Injection for the Treatment of Gastroenteropancreatic Neuroendocrine Tumors 2Ipsen Announces the FDA Approval of Somatuline® Depot® (lanreotide) Injection for the Treatment of Gastroenteropancreatic Neuroendocrine Tumors 3Ipsen Announces the FDA Approval of Somatuline® Depot® (lanreotide) Injection for the Treatment of Gastroenteropancreatic Neuroendocrine Tumors 4Ipsen Announces the FDA Approval of Somatuline® Depot® (lanreotide) Injection for the Treatment of Gastroenteropancreatic Neuroendocrine Tumors 5Ipsen Announces the FDA Approval of Somatuline® Depot® (lanreotide) Injection for the Treatment of Gastroenteropancreatic Neuroendocrine Tumors 6Ipsen Announces the FDA Approval of Somatuline® Depot® (lanreotide) Injection for the Treatment of Gastroenteropancreatic Neuroendocrine Tumors 7Ipsen Announces the FDA Approval of Somatuline® Depot® (lanreotide) Injection for the Treatment of Gastroenteropancreatic Neuroendocrine Tumors 8
... Pa., Sept. 23 Environmental,Tectonics Corporation,s (Amex: ... announced the sale and placement of two BARA-MED(R) ... The,Shoreline Facility was established in Corpus Christi, Texas, ... care medical center in the region. The,facility offers ...
... Premacure to Initiate Phase II Trial for IPLEX(TM) ... UPPSALA, Sweden, Sept. 23 ,Insmed Inc. (Nasdaq CM: ... and Premacure AB, a biopharmaceutical company,dedicated to the ... due to premature birth, today noted the presentation ...
... Vein Recognition Device Helps BayCare Accurately,Identify Patients, ... Patient Privacy, SUNNYVALE, Calif., Sept. 23 ... leading supplier of innovative computer products,including hard ... announced that BayCare Health System, the largest ...
Cached Biology Technology:Environmental Tectonics Corporation Announces a Multiple Sale for Monoplace Hyperbaric Chambers 2Environmental Tectonics Corporation Announces a Multiple Sale for Monoplace Hyperbaric Chambers 3Insmed and Premacure Cite Study Results Demonstrating Potential Effectiveness of IPLEX(TM) in Preventing Blindness in Premature Infants 2Insmed and Premacure Cite Study Results Demonstrating Potential Effectiveness of IPLEX(TM) in Preventing Blindness in Premature Infants 3Insmed and Premacure Cite Study Results Demonstrating Potential Effectiveness of IPLEX(TM) in Preventing Blindness in Premature Infants 4Insmed and Premacure Cite Study Results Demonstrating Potential Effectiveness of IPLEX(TM) in Preventing Blindness in Premature Infants 5Fujitsu PalmSecure and HT Systems' PatientSecure Selected by BayCare Health System to Protect Patient Confidentiality and Prevent Medical Identity Theft 2Fujitsu PalmSecure and HT Systems' PatientSecure Selected by BayCare Health System to Protect Patient Confidentiality and Prevent Medical Identity Theft 3Fujitsu PalmSecure and HT Systems' PatientSecure Selected by BayCare Health System to Protect Patient Confidentiality and Prevent Medical Identity Theft 4Fujitsu PalmSecure and HT Systems' PatientSecure Selected by BayCare Health System to Protect Patient Confidentiality and Prevent Medical Identity Theft 5
(Date:12/17/2014)... 15, 2014  HITLAB SM announced today ... audit to confirm its adherence to current U.S. ... enables HITLAB to conduct regulated smart device and ... for patient safety and research quality. ... quality, and delivery with innovative technology," said ...
(Date:12/17/2014)... DENVER , Dec. 16, 2014  Automation ... is this more evident than at international borders. ... secure document scanners have allowed veteran travelers to ... Automated Passport Control (APC) Kiosks at an increasing ... the globe. According to ...
(Date:12/11/2014)... 09, 2014 Research and Markets ... ( http://www.researchandmarkets.com/research/9ql3kr/biometrics_market ) has announced the addition ... report to their offering. ... the adoption of multimodal biometric systems. Multimodal biometric ... individual for verification and identification purposes. This helps ...
Breaking Biology News(10 mins):HITLAB Completes GCP Audit for FDA Smart Device & App Compliance 2Automated Border Control (ABC) Transforms the Global Travel Experience With More Than 2500 ABC eGates and APC Kiosks Deployed In Airports, Seaports, and Land Borders Worldwide 2Biometrics Market in India 2015-2019: Key Vendors are 3M Cogent, NEC, Safran and Suprema 2
... For the first time in nearly 50 years, ... rediscovered in the San Bernardino National Forest,s San Jacinto ... suitability of sites to re-establish frogs and scientists from ... natural history expedition both rediscovered the rare mountain yellow-legged ...
... , VILNIUS, Lithuania, July 27 ... identification technologies provider, announced today their inclusion as the core ... Issuance System. MegaMatcher SDK multi-biometric technology ... PT. Berca Hardayaperkasa, to develop a distributed passport ...
... - Temperature explains much of why cold-blooded organisms ... at higher latitudes than at lower latitudes, according to ... the National Academy of Sciences ( PNAS ) ... Santiago Salinas, both of Stony Brook University,s School of ...
Cached Biology News:Biologists rediscover endangered frog population 2Biologists rediscover endangered frog population 3Biologists rediscover endangered frog population 4Indonesia Now Using MegaMatcher Biometric Technology in Integrated Passport Issuance System 2Indonesia Now Using MegaMatcher Biometric Technology in Integrated Passport Issuance System 3Indonesia Now Using MegaMatcher Biometric Technology in Integrated Passport Issuance System 4Warmer environment means shorter lives for cold-blooded animals 2
... HT is designed for extended ... HT is ideal for applications ... primer extension assays. The iEMS ... a one-cabinet model, allowing the ...
... Source: Pseudomonas atlantica Description: Beta-Agarase is ... fragments from low-melting-temperature agarose. Beta-Agarase digests ... Tested User Friendly Properties: Heat Inactivation: ... Da Optimum pH: 6.5 Purity: Tested ...
iEMS Thermal Microplate Holder...
Improve inventory management with stainless steel red cell canisters; ensure product safety with CO2 or LN2 backup systems. A complete solution for your blood banking needs....
Biology Products: