KC Scientists Awarded $150,000 to Further Research on Childhood Diseases

KANSAS CITY, MO – BioNexus KC awarded $50,000 each to three area researchers to support research focused on genetic diseases affecting children such as autism, jaundice, and intestinal inflammation. The funds to support the projects are provided by the Paul Patton Trust, Ted C. McCarter, William Evans, Jr., and Bank of America, N.A. Trustees.

 “We are proud to work with the Paul Patton Charitable Trust and Bank of America to find and fund crucial pediatric research for over ten years,” said Dr. Keith Gary, Vice President of BioNexus KC. “The funding supports new approaches to finding cures for genetic diseases that primarily impact children. The data generated through these projects better positions the grantees in competing for larger federal grants to continue research programs.”

Below are this year’s grant recipients and a brief description of their research programs.

Role of Specific Receptor Mutations in a life-threatening inflammatory bowel disease in Premature Infants
A team of investigators led by Dr. Venkatesh Sampath at Children’s Mercy will research severe intestinal inflammation in premature infants called necrotizing enterocolitis (NEC). Infants that develop NEC have a high mortality rate of 20-35%. Studies by this team had initially identified a gene called SIGIRR as a responsible gene for NEC. In this project, the investigators will examine how mutations in this gene can alter the ability of intestinal bacteria to cause NEC using a novel mouse model.

“We will determine how mutations in this gene, SIGIRR contribute to NEC and whether probiotics (good bacteria) and other anti-inflammatory metabolites can prevent NEC in an infant’s intestine” said Dr. Sampath. “This research is part of our long-term goal to prevent this devastating disease by implementing genetic-screening and targeted use of novel therapies.”

Establishing a Novel Mouse Model of an Autism Spectrum Disorder
According to a recent CDC autism report, the rates of autism are rising among children in the U.S. Drs. Irfan Saadi and Hiroshi Nishimune from the University of Kansas Medical Center will study the cellular and molecular mechanisms causing behavior defects and nerve cell degeneration resulting from a mutation in the mouse Specc1l gene.  Mutations in this gene have been identified in patients with Opitz G/BBB syndrome and Teebi Hypertelorism syndrome (THS) – two autism spectrum disorders.

Dr. Saadi has generated a mouse model to specifically research reduced SPECC1L levels that produce hyperactivity and repetitive behaviors that often accompany brain cell loss. “In a recent publication looking at gene expression differences in autistic brains, SPECC1L expression was observed to be reduced significantly. Thus, our mouse model represents an excellent tool to study the behavioral deficits of Opitz G/BBB and Teebi Hypertelorism syndromes, and perhaps autism in general,” said Dr. Saadi. Drs. Saadi and Nishimune will also be collaborating with investigators at Children’s Mercy Hospital to identify more patients with SPECC1L mutations to better understand associated behavioral deficits. “Studying genes responsible for autism spectrum disorders will help us understand autism at-large.”

Protective and susceptibility factors – brain responses to abnormally high Bilirubin levels in Jaundice
Dr. Sean Riordan, Children’s Mercy, will research jaundice, the yellowing of the skin and eyes that occurs with high levels of the yellow pigment bilirubin. While jaundice occurs in 60–80% of all newborns, a small portion of infants develop severe jaundice and are at risk of developing brain damage.  High levels of bilirubin can move into the brain causing a form of cerebral palsy.

“These studies will leverage our ability as one of the few laboratories in the world able to measure free bilirubin in the blood,” said Dr. Riordan. “The work described in this project has potential to expose targets for new drug therapies to augment existing treatments and improve outcomes of newborns experiencing severe jaundice.”

Information about the Patton Trust Research Development Grants, including eligibility, review criteria and application procedures, can be found on the BioNexus KC website.

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About BioNexus KC:
Through its Research Development Grants program, BioNexus KC manages a variety of individual grants for area corporations and trusts, helping them identify proposals with the best scientific, medical and technical merit.  Research grants are awarded to generate initial results and stimulate the submission of major multidisciplinary research proposals to government or private agencies. BioNexus KC’s Research Development Grants program includes proposal review, evaluation by subject matter experts, written reviews for all applicants, and post-award management.

Media Inquiries:
Dr. Keith Gary
BioNexus KC – Vice President
kgary@bionexuskc.org
816-753-7700

Shaylee Yount
BioNexus KC – Communications and Events Manager
syount@bionexuskc.org
816-753-7700

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