HOME >> BIOLOGY >> NEWS
A protein's role in progressive renal disease

Focal segmental glomerulosclerosis (FSGS) made a brief media splash last year when a kidney transplant forced NBA superstar Alonso Mourning into early retirement. Mourning's condition elicited a flood of calls from fans offering their kidneys, but most people with kidney disease are not so lucky.

Some 56,000 patients await transplants; many have waited over five years. FSGS, which underlies about 25% of the 60,000 kidney-related deaths each year, causes progressive kidney failure.

FSGS commonly occurs as an outgrowth of various primary disorders, including obesity, HIV infection, diabetes, and hypertension. Though it's not clear what causes FSGS, this form of renal pathology is becoming more common.

By using the genes underlying inherited forms of FSGS as probes, scientists hope to uncover the mechanisms that unleash the disease and to find ways to stem the damage.

Mutations in the ACTN4 gene, which encodes a protein called a-actinin-4, cause an inherited form of FSGS. The protein normally remodels actin filaments, the primary structural component of muscle and cytoskeleton.

Having a single mutated copy of the gene can cause FSGS in humans, though it is unclear how. In this issue of PLoS Biology, Martin Pollak and his colleagues at Brigham and Women's Hospital at Harvard Medical School use a three-pronged approach to figure out how the defective protein wreaks renal havoc and how these physiological changes lead to FSGS.

Using biochemical analysis, cell-based studies, and a newly developed "knockin" mouse model, the researchers report two possible mechanisms of initiating disease.

Aggregation of a-actinin-4 and the toxic affects of aggregation could injure kidney cells, or loss of a-actinin-4 function caused by rapid degradation of the protein could produce injury.

Pollak and colleagues argue that both factors likely play a role.


'"/>

Contact: Susanne DeRisi
sderisi@plos.org
415-624-1207
Public Library of Science
15-Jun-2004


Page: 1

Related biology news :

1. Optimizing proteins death domain halts leukemia in laboratory study
2. Findings suggest need for new view of p53 cancer proteins interaction with DNA
3. Rb proteins role in retina development is key to understanding devastating eye cancer
4. Researchers view proteins structural changes in real time
5. Misfolding the key to proteins ability to kill brain cells
6. Researchers solve killer proteins crime
7. Biologists discover proteins impace on plant-water balance
8. Penn researchers proteins link to leukemia
9. Gene mutation causes progressive changes to cell structure in children with Progeria
10. Researchers develop blood test that can detect genetic changes in progressive breast cancer
11. Lions share of the best of Asias progressive minds in science and medicine convene

Post Your Comments:
*Name:
*Comment:
*Email:


(Date:7/2/2020)... ... July 01, 2020 , ... ... 1a findings of Neihulizumab, a biologic for the treatment of steroid-refractory acute graft-versus-host ... by hemato-oncologist Dr. Paul J Martin of the Fred Hutchinson Cancer Research Center, ...
(Date:6/28/2020)... BASEL, Switzerland (PRWEB) , ... ... ... leading provider of enterprise software solutions for biopharmaceutical R&D, today announced that ... oncology company developing innovative, full-length multispecific antibodies (Multiclonics®), to support their translational ...
(Date:6/23/2020)... ... June 23, 2020 , ... ... healthcare, and Renovagen Ltd, a UK supplier and manufacturer of innovative portable renewable ... testing operations in the fight against the COVID-19 pandemic in Zambia. , One ...
Breaking Biology News(10 mins):
(Date:7/31/2020)... ... July 29, 2020 , ... The SDX® Respiratory ... 16 countries, has reached its 20th anniversary of worldwide use. Introduced in the ... top universities including University of Pennsylvania, University of Michigan, University of Maryland, University ...
(Date:7/18/2020)... ... ... After research model surgery and catheter implantation, the long journey of catheter maintenance ... a successful study, while protecting and accessing the catheter or device post-surgery is often ... Envigo in a live webinar on Wednesday, August 5, 2020 ...
(Date:7/18/2020)... ... July 17, 2020 , ... ... for the life sciences and food industries, is pleased to announce that Charles ... Research – Business Development. , Charles is an accomplished and results-driven sales executive ...
(Date:7/10/2020)... ... July 09, 2020 , ... In most research using the ... pigment formation in zebrafish embryos, maintaining optical transparency to facilitate microscopic imaging. Over ... been using the zebrafish model to investigate the causes of leukaemia and its ...
Breaking Biology Technology:
Cached News: