BILL ANALYSIS                                                                                                                                                                                                    






                                 SENATE HEALTH
                               COMMITTEE ANALYSIS
                        Senator Elaine K. Alquist, Chair


          BILL NO:       SJR 23                                       
          S
          AUTHOR:        Simitian                                     
          J
          AMENDED:       April 29, 2010                               
          R              
          HEARING DATE:  May 5, 2010                                 
          CONSULTANT:                                                 
          2
          Orr/cjt                                                     
          3              
                                     SUBJECT
                                         
              Mitochondrial disease: federal support for research

                                     SUMMARY  

          Urges the President and Congress to amend federal statutes  
          to support research for mitochondrial disease. 


                             CHANGES TO EXISTING LAW  

          Existing federal law:
          Establishes the National Institutes of Health (NIH) within  
          the U.S. Department of Health and Human Services for the  
          purposes of conducting and supporting medical research.

          Existing state law:
          Establishes the Department of Public Health within the  
          California Health and Human Services Agency to, among other  
          things, research various public health problems, collect  
          and manage relevant health data, and award grants.
          
          This resolution:
          Urges the President and Congress to take action necessary  
          to amend federal statutes to support research for  
          mitochondrial disease. 

          Makes findings and declarations regarding mitochondrial  
          diseases.
                                                         Continued---



          STAFF ANALYSIS OF SENATE JOINT RESOLUTION 23          Page  
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                                  FISCAL IMPACT
                                         
          This resolution is keyed nonfiscal.




                            BACKGROUND AND DISCUSSION  

          The intent of this resolution is to urge the President and  
          Congress to significantly expand support for mitochondrial  
          research and improve our understanding of mitochondrial  
          dysfunction by passing H.R. 3502 and S. 2858, related to  
          mitochondrial disease research and treatment.  

          Mitochondrial disease
          Mitochondria are cell components that generate energy for  
          the cell. Each cell in the body contains anywhere from  
          dozens to hundreds of mitochondria, which are responsible  
          for creating more than 90 percent of the energy needed by  
          the body to sustain life and support growth. According to  
          the NIH, mitochondrial diseases are caused by abnormal  
          genes that lead to flawed proteins or other molecules in  
          the mitochondria. When they fail, less and less energy is  
          generated within the cell. Cell injury and cell death can  
          follow. 

          Depending on which cells are affected, people with  
          mitochondrial diseases may have muscle weakness and pain,  
          digestive problems, heart disease, seizures and many other  
          symptoms. Hardest hit are organs and tissues that need a  
          lot of energy, like muscles, brain, heart, kidneys and  
          liver. When the energy supply slumps, cells can become  
          damaged or destroyed.  There is no treatment or cure for  
          any of these rare diseases.

          There are estimated to be tens of thousands of people  
          affected by mitochondrial disease in the U.S. Because the  
          symptoms vary widely, mitochondrial diseases are often  
          mistaken for other conditions. There are dozens of types of  
          mitochondrial diseases, with each affecting less than 1 in  
          1,500 people.  The various types are caused by alterations  
          in different genes, leading to worn-down cells in different  
          parts of the body. These diseases affect both children and  
          adults, and sometimes lead to early death.




          STAFF ANALYSIS OF SENATE JOINT RESOLUTION 23          Page  
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          Current bills in Congress
          H.R. 3502 (McDermott) was introduced in the U.S. House of  
          Representatives in July 2009, and seeks to amend the Public  
          Health Service Act to establish an Office of Mitochondrial  
          Medicine (Office) at the National Institutes of Health.  S.  
          2858 (Boxer) was introduced in the U.S. Senate in December  
          2009, and is nearly identical to H.R. 3502. 

          The purpose of the Acts is to promote research aimed at  
          improved understanding of mitochondrial disease and  
          dysfunction and the development of treatments for  
          mitochondrial disease. The Office would be responsible for  
          developing a plan to improve coordination of research  
          related to mitochondrial medicine among the national  
          research institutes and between the NIH and outside  
          researchers. The Office would provide training to  
          scientists, health professionals, and health care providers  
          on research and diagnosis of mitochondrial disease and  
          dysfunction, and will annually award a minimum number of  
          grants to further these purposes and to establish  
          Mitochondrial Medicine Centers of Excellence.  The Office  
          would also establish a national registry for the  
          maintenance and sharing for research purposes of medical  
          information collected from patients with mitochondrial  
          disease and dysfunction, and a national biorepository to  
          collect tissues and DNA.  

          

          National Institutes of Health 
          The NIH is the federal focal point for biomedical research.  
          The Office of Rare Diseases Research (ORDR) was established  
          in 1993 within the Office of the Director of the NIH to  
          coordinate and support rare diseases research and to  
          provide information on rare diseases. A rare disease is any  
          disease or condition affecting fewer than 200,000 persons  
          in the U.S. According to the ORDR, there are over 6,800  
          rare diseases known today, affecting an estimated 25 to 30  
          million people in the U.S. 
          
          According to the Secretary of Health and Human Services,  
          many institutes at the NIH are currently supporting  
          research relevant to mitochondrial function and  
          dysfunction. NIH has committed to establish a mitochondrial  
          research initiative that would involve all NIH institutes,  




          STAFF ANALYSIS OF SENATE JOINT RESOLUTION 23          Page  
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          and has awarded grant funding for mitochondrial research. 

          Prior legislation
          ACR 25 (Miller), Chapter 15, Statutes of 2009, proclaims  
          the third week in September as Mitochondrial Disease  
          Awareness Week.

                                    POSITIONS  


          Support:  384 individuals

          
          Oppose:  None


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