Creutzfeldt Jakob Disease News and Research

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Creutzfeldt–Jakob disease or CJD (sometimes incorrectly referred to as mad cow disease) is a degenerative neurological disorder (brain disease) that is incurable and invariably fatal. It is the most common among the types of transmissible spongiform encephalopathy found in humans.

CSL Behring presents Hizentra study data against PI at AAAAI meeting

Data presented by CSL Behring today suggest that treatment with higher dose Hizentra correlates with reduced risk of infection and missed school or work among patients with primary immunodeficiencies.

22 Mar 2011

New research may shed light on possible treatments for prion diseases

New research by Chongsuk Ryou, researcher at the UK Sanders-Brown Center on Aging and professor of microbiology, immunology and molecular genetics in the UK College of Medicine, may shed light on possible treatments for prion diseases.

15 Mar 2011

First disease-specific biomarker for sporadic Creutzfeldt-Jakob disease identified

Neena Singh, MD, PhD and colleagues at Case Western Reserve University School of Medicine have identified the first disease-specific biomarker for sporadic Creutzfeldt-Jakob disease (sCJD), a universally fatal, degenerative brain disease for which there is no cure. sCJD is one of the causes of dementia and typically leads to death within a year of disease onset.

10 Mar 2011

Grifols receives FDA approval for Alphanate to treat vCJD

Grifols, a global healthcare company and biopharmaceutical manufacturer based in Barcelona, Spain today announced that it received approval from the US Food and Drug Administration for revised labeling for Alphanate Antihemophilic Factor/von Willebrand Factor Complex indicating that certain manufacturing steps have been shown to reduce the infectivity of a experimental TSE agent that is a model for variant Creutzfeldt Jakob Disease.

5 Mar 2011

FDA approves sBLA for CSL's Hizentra to treat PI

CSL Behring announced today that the U.S. Food and Drug Administration has approved a supplemental Biologics License Application to extend the shelf life of Hizentra, Immune Globulin Subcutaneous, 20% Liquid, from 24 months to 30 months.

24 Feb 2011

Aminothiazoles as therapeutic leads for prion diseases

Scientists who examined more than 10,000 chemical compounds during the last year in search of potential new drugs for a group of untreatable brain diseases, are reporting that one substance shows unusual promise.

24 Feb 2011

How Huntington's disease proteins move from cell to cell

One bad apple is all it takes to spoil the barrel. And one misfolded protein may be all that's necessary to corrupt other proteins, forming large aggregations linked to several incurable neurodegenerative diseases such as Huntington's, Parkinson's and Alzheimer's.

19 Feb 2011

FDA grants CSL Behring marketing approval for Corifact in treatment of congenital FXIII deficiency

CSL Behring announced today that the U.S. Food and Drug Administration (FDA) has granted marketing approval for Corifact™, Factor XIII Concentrate (Human), for the routine prophylactic treatment of congenital factor XIII (FXIII) deficiency. Corifact, already available for use in 12 countries throughout the world under the trade name Fibrogammin®- P, is the first and only FXIII concentrate approved in the U.S.

19 Feb 2011

Researchers identify normal movement mechanisms of PrPC in brain

Researchers at the University of California, San Diego School of Medicine have identified the motors that move non-infectious prion proteins (PrPC) - found within many mammalian cells - up and down long, neuronal transport pathways.

18 Feb 2011

Blood test for screening mad cow disease to come soon: Study

UK scientists have developed a new blood test that can diagnose cases of variant Creutzfeldt-Jakob Disease (vCJD), a rare, fatal brain disease caused by proteins known as prions. These prions are also responsible for the mad cow disease, or bovine spongiform encephalopathy (BSE). This test could in near future be used to identify contaminated blood and prevent transmission of the disease during blood transfusions, the researchers said.

6 Feb 2011

Airborne prions can induce mad cow disease: Research

Airborne prions are also infectious and can induce mad cow disease or Creutzfeldt-Jakob disorder. This is the surprising conclusion of researchers at the University of Zurich, the University Hospital Zurich and the University of Tübingen. They recommend precautionary measures for scientific labs, slaughterhouses and animal feed plants.

14 Jan 2011

Plasminogen may serve as new target to treat prion diseases

Scientists at the University of Kentucky have discovered that plasminogen, a protein used by the body to break up blood clots, speeds up the progress of prion diseases such as mad cow disease.

11 Jan 2011

Prions responsible for BSE can survive in new host environment

Scientists from the Florida campus of The Scripps Research Institute have shown that prions, bits of infectious protein that can cause fatal neurodegenerative disease such as bovine spongiform encephalopathy or "mad cow disease," have the ability to adapt to survive in a new host environment.

17 Dec 2010

Talecris initiates clinical trial to evaluate 2 doses of PROLASTIN-C for emphysema due to AAT deficiency

Talecris Biotherapeutics, Inc. announced today the initiation of a clinical trial evaluating the safety and the pharmacokinetic profile of two doses of PROLASTIN®-C (Alpha(1)-Proteinase Inhibitor [Human]) (A1PI), a therapy indicated for chronic augmentation and maintenance in adults with emphysema due to alpha(1)-antitrypsin (AAT) deficiency. AAT deficiency is a rare, genetic disorder in which low levels of the alpha(1) protein circulating in the lungs can increase an individual's risk of developing emphysema.

10 Dec 2010

NIAID researchers develop new method to detect prion diseases

A fast test to diagnose fatal brain conditions such as mad cow disease in cattle and Creutzfeldt-Jakob disease in humans could be on the horizon, according to a new study from National Institutes of Health scientists. Researchers at NIH's National Institute of Allergy and Infectious Diseases (NIAID) have developed a highly sensitive and rapid new method to detect and measure infectious agents called prions that cause these diseases.

3 Dec 2010

Scientists find new protein to break-up blood clots in prion diseases

The joy of a juicy hamburger could make a comeback thanks a new discovery by scientists from the University of Kentucky. In a new research report in the December 2010 print issue of The FASEB Journal, scientists found that a protein our body uses to break up blood clots speeds up the progress of prion diseases. This substance, called plasminogen, is a new drug target for prion diseases in both humans and animals.

2 Dec 2010

Talecris announces publication of A1PI studies for AAT deficiency in Respiratory Research journal

Talecris Biotherapeutics announced today the publication of combined data from two studies demonstrating that augmentation therapy with Alpha(1)-Proteinase Inhibitor (Human) (A1PI) significantly reduces lung tissue loss in patients with emphysema related to Alpha(1)-antitrypsin (AAT) deficiency.

15 Nov 2010

Talecris announces launch of PROLASTIN-C in Canada

Talecris Biotherapeutics announced today the Canadian launch of PROLASTIN®-C (Alpha1-Proteinase Inhibitor [Human])(A1PI), a more concentrated and purified formulation of PROLASTIN®, the only approved alpha1-augmentation therapy in Canada for more than 20 years. PROLASTIN-C delivers twice the active protein per milliliter as PROLASTIN, cutting the infusion volume and time in half when given at the recommended rate of 0.08 mL/kg/min.

3 Nov 2010

Baxter announces launch of GLASSIA in the U.S.

Baxter International Inc. today announced the commercial launch of GLASSIATM [Alpha1-Proteinase Inhibitor (Human)] in the United States. GLASSIATM is the first available ready-to-use liquid alpha1-proteinase inhibitor (Alpha1-PI) and is indicated as a chronic augmentation and maintenance therapy in adults with emphysema due to congenital deficiency of alpha-1 antitrypsin (AAT), an under-diagnosed hereditary condition characterized by a low level of alpha-1 protein in the blood.

From Baxter International Inc. 26 Oct 2010

Talecris announces publication of PROLASTIN-C study results for AAT deficiency in BML journal

Talecris Biotherapeutics today announced the publication of results from a study showing that PROLASTIN-C (Alpha1-Proteinase Inhibitor [Human]) (A1PI) is as effective as PROLASTIN (Alpha1-Proteinase Inhibitor [Human]) in raising levels of alpha-1 protein in patients with alpha1-antitrypsin (AAT) deficiency.

22 Oct 2010