Geometry.Net - Science: Mitochondrial Genetics

ERIC A. SCHON
ERIC A. SCHON, Ph.D. mitochondrial genetics AND THE MOLECULAR BASIS OF HUMAN MITOCHONDRIAL DISEASE
http://cpmcnet.columbia.edu/dept/genetics/faculties/Schon.html

Extractions: Mitochondria are unique among the constituents of the eukaryotic cell in that they are semi-autonomous organelles that contain their own genetic machinery. As such, they operate under the dual genetic controls of nuclear DNA (nDNA) and mitochondrial DNA (mtDNA). Mitochondrial genetics differs markedly from mendelian genetics, because first, mitochondria are inherited exclusively from the mother, and second, there are hundreds or thousands of mitochondria (and mtDNAs) per cell. In addition, organellar division and mtDNA replication are stochastic processes unrelated to the cell cycle, and mtDNA gene organization, DNA replication, RNA transcription, and protein translation all have a prokaryotic "look" about them. This latter feature is no surprise, given that mitochondria were once bacteria that were taken up by the proto-eukaryotic cell early in evolution. Biochemically, the most relevant aspect of mitochondrial function is the production of oxidative energy via the respiratory chain and oxidative phosphorylation. Mitochondrial diseases have turned out to be equally unusual. There are maternally-inherited, mendelian-inherited, sporadic, and even environmentally induced mitochondrial disorders, most of which are either severely debilitating or fatal. We are studying the molecular basis of a number of these diseases, most of which are heteroplasmic (i.e. both mutant and wild-type mtDNAs coexist in varying proportions in the same patient), using a novel tissue culture system called "r
LTU: Microbiology: Dictyostelium Mitochondrial Genetics Laboratory
Dr. Christian Barth Welcome to the Dictyostelium mitochondrial geneticsPage. Location mitochondrial genetics Laboratory Department
http://www.latrobe.edu.au/microbiology/barth.html

Extractions: E-mail: C.Barth@latrobe.edu.au Click here to see Dr Christian Barth's CV Research Interests: Mitochondria are found in almost all eukaryotic cells. They are the powerhouse of the cell, generating energy in form of ATP, and have other important functions in haem and lipid biosynthesis, intracellular signal transduction and programmed cell death. According to the endosymbiotic hypothesis Although the number of proteins still encoded in the mitochondrial genome is relatively small, the organellar genome has to be maintained, replicated and transcribed, and the encoded gene products have to be synthesised on ribosomes in the mitochondrial matrix. The genetic processes involved are quite different from those in the nucleus and in the cytoplasm of the eukaryotic host - in fact they are strikingly similar to the genetic processes found in bacteria. However, the proteins that mediate mitochondrial replication, transcription and protein synthesis are now all nuclear-encoded.
Basic Genetics
The following article from the MDA has a great explanation of mitochondrial geneticsand can be found at http//www.mdausa.org/publications/Quest/q64mito.html.
http://www.umdf.org/mitodisease/genetics.html

Extractions: Sporadic Introduction and Definition Unfortunately, the multiple diseases classified as mitochondrial disorders are inherited in different manners. In fact, nearly every inheritance "model" known has been demonstrated to occur in mitochondrial disease. However, most mitochondrial disorders known to date are inherited in either an autosomal recessive or maternal manner. The model of inheritance is important in that it can be helpful in answering the following questions: Are other family members, either existing or not yet born, at risk for developing mitochondrial disease? What is the risk (percentage)? When will other affected relatives become ill? Will other affected relatives be as sick as my child/myself? Possibly even sicker? What kinds of problems/diseases might other affected relatives suffer from?
NeuroCAST - Genetics And Diagnosis Of Mitochondrial Disorders
This article is a brief review of mitochondrial genetics, disorders, and theirdiagnosis for neurologists, geneticists, genetic counselors, and other
http://www.neurocast.com/site/content/sessions_02_2002.asp

Extractions: Mitochondria are the intracellular organelles that supply most of a cell's energy needs by producing adenosine triphosphate (ATP) through oxidative phosphorylation. More recently, mitochondria have also been found to play a central role in programmed cell death, or apoptosis. In addition to these well-known tasks, mitochondria are also responsible for a variety of other metabolic functions specific to the almost 250 different cell types in the human body. Mitochondria are unique organelles in that they contain their own DNA (mtDNA), which is distinct from the DNA in the cell nucleus (nDNA). Thus, proper mitochondrial function depends on the coordinated expression of both the nuclear and mitochondrial genomes and therefore, mitochondrial dysfunction can arise from mutations in either genome (Figure 1). This article is a brief review of mitochondrial genetics, disorders, and their diagnosis for neurologists, geneticists, genetic counselors, and other healthcare professionals involved in the care of people with complex neurological diseases that may be due to mitochondrial dysfunction. Figure 1.
Mitochondrial Genetics
English title mitochondrial genetics Swedish title Mitokondriell genetik Projectleader NilsGöran Larsson All projects by this author Institution Department
http://research.kib.ki.se/e-uven/show_project.cfm?projects_no=K1974
Mitochondrial Genetics
How does a murine cell cope with loss of mitochondrial DNA? The Howis the mitochondrial transcription machinery regulated? How
http://www.mednut.ki.se/research/ngl/mito.html

Extractions: How does a murine cell cope with loss of mitochondrial DNA? The answer is different depending on cell type, but the end is always the same; cell death. The group is situated at the Department of Medical Nutrition at Karolinska Institutet in Stockholm, the beautiful capital of Sweden. From being rather small, the team has expanded significantly during the last couple of years and now consists of five postdoctoral fellows, eight graduate students, one undergraduate student and one technical assistant. The research carried out in the laboratory is very diverse, but the main focus is on mitochondrial transcription and what happens when it is impaired. We have to date primarily worked with the nuclear encoded mitochondrial transcription factor A, Tfam, and what happens when this protein is lacking. Tfam is essential for mtDNA maintenance and loss of the Tfam protein leads to a complete loss of mtDNA. Oxidative phosphorylation, OXPHOS; is depending on mitochondrial encoded subunits to function, so the mitochondria are not able to produce ATP if mtDNA is gone. We have knocked out the Tfam gene from the entire mouse as well as in individual tissues such as skeletal muscle, heart, pancreas and brain. In all instances the affected cells cannot survive without the Tfam protein. For how long depends on the affected tissue and how early the knockout occurs.
Mitochondrial Genetics Publications
Increased mitochondrial mass in mitochondrial myopathy mice. Larsson NG. Leber hereditaryoptic neuropathy A nuclear solution of a mitochondrial problem.
http://www.mednut.ki.se/research/ngl/pub.html

Extractions: List of publications Wredenberg A, Wibom R, Wilhelmsson H, Graff C, Wiener HH, Burden SJ, Oldfors A, Westerblad H, Larsson NG. Increased mitochondrial mass in mitochondrial myopathy mice. PNAS 2002 Nov 12; 99 (23):15066-15071 Larsson NG. Leber hereditary optic neuropathy: A nuclear solution of a mitochondrial problem. Ann Neurol 2002 Nov; 52(5):529-530 ... A single mouse gene encodes the mitochondrial transcription factor A and a testis-specific nuclear HMG-box protein. Nat Genet. 1996 Jul;13(3):296-302.
Unique Challenges Mitochondrial Genetics Biology Of The
Advanced Human Genetics. mitochondrial genetics (D. P. Nierlich)
http://www.genetics.ucla.edu/home/class/1-18-01.pdf
Genetics Department
14. Hisheh,S., Schmitt,L and Westerman, M. (1998). Indonesian biogeographymitochondrial genetics of the fruit bat, Eonycteris spelaea. Biol.
http://www.latrobe.edu.au/genetics/staff/westerman/homepage/

Extractions: About La Trobe Faculties Campuses Research ... International HomePage A.Prof. Michael Westerman BSc(Hons), PhD, DSc. (Birmingham). Reader in Marsupial Molecular Evolution My research interests centre primarily on the reconstruction and understanding of the phylogenetic relationships of marsupials using nuclear and mitochondrial DNA sequence data as well as chromosome banding and painting techniques. Molecular Phylogenetics of Dasyurids. The family dasyuridae includes over 65 species of carnivorous/insectivorous marsupials from Australia and New Guinea. They occupy almost all Australasian habitats from rainforests to the arid interior. Ongoing collaboration with Dr C.Krajewski (SIU, Carbondale,Illinois) is aimed at reconstructing phylogenetic relationships of all species using DNA sequences to help clarify palaeobiological questions as well as the evolution of reproductive strategies in the group. Currently we are utilising a series of nuclear genes to complement our extensive mitochondrial database for dasyurid species
Mayo Clinic - Isaya Lab - Mitochondrial Genetics
Isaya Lab mitochondrial genetics.
http://www.mayo.edu/research/isayalab/

Extractions: The Lab Members Mitochondrial oxidative phosphorylation (OXPHOS) is the main source of ATP for the eukaryotic cell. OXPHOS, however, is also the main source of reactive oxygen species (ROS). As it turns out, OXPHOS is the most immediate target of ROS attack because biomolecules essential for OXPHOS activity such as mitochondrial DNA and iron-sulfur proteins are exquisitely susceptible to oxidative damage . This situation is exacerbated by the fact that OXPHOS biogenesis requires a continuous supply of iron-sulfur clusters and heme , which in turn depends on the availability of Fe , and redox-active iron can catalyze production of hydroxyl radical , OH , the most reactive oxygen species known. Our lab is interested in the mechanisms that enable the cell to exploit the energy efficiency of OXPHOS in spite of the risks inherent in ROS production. In particular, we are interested in the role played by components of the mitochondrial protein import machinery in OXPHOS maintenance. Another area of great interest to our lab are mitochondrial proteins involved in
Mitochondrial Genetics
RFLP analysis show that the phenotypic modifications and male sterility are associatedwith a pure Arabidopsis or a recombined mitochondrial genome containing
http://www.vbiol.slu.se/tema/growth/CMS/21.html

Extractions: Ingrid Eriksson Regulation of floral organogenesis and CMS in Brassica Brassica napus Arabidopsis thaliana somatic cybrids as a tool for investigating floral development and cytoplasmic male sterility (CMS) From protoplast fusions between Arabidopsis thaliana and Brassica napus (oil seed rape) alloplasmic lines have been produced. Several lines display modified and aberrant flower phenotypes with distorted petals and anthers. Most of these lines are also male sterile. Genetic characterizations of mitochondria using RFLP analysis show that the phenotypic modifications and male sterility are associated with a pure Arabidopsis or a recombined mitochondrial genome containing mtDNA from both species. Recurrent backcrosses are performed to obtain a stable nuclear Brassica napus genome. The aim is to find mitochondrial genes involved in the CMS trait and flower development. The availability of the complete Arabidopsis thaliana mitochondrial DNA sequence facilitates studies of mitochondrial gene expression by run-on and Northern experiments.
Mitochondrial Genetics
English title. mitochondrial genetics. Swedish title
http://research.kib.ki.se/e-uven/public/K1974.html

Extractions: Larsson NG, Wang J, Wilhelmsson H, Oldfors A, Rustin P, Lewandoski M, Barsh GS, Clayton DA. Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice.Nature Genetics 1998:18:231-236. Larsson NG, Garman JD, Oldfors A, Barsh GS, Clayton DA. A single mouse gene encodes the mitochondrial transcription factor A and a testis-specific nuclear HMG-box protein.Nature Genetics 1996:13:296-302. Project staff Caroline Graff Postdoc Jose Silva Postdoc Jianming Wang PhD student Anja Rantanen PhD studen Hong Li PhD studen Mats Ekstrand PhD studen Carita Holm Technichian Entered Updated
Flower Development & Mitochondrial Genetics
Lines displaying modified and aberrant flower. phenotypes with distortedpetals and anthers. Normal Brassica napus. CMSline with shrunken anthers.
http://www.vbiol.slu.se/tema/growth/CMS/lines
Mitochondrial Genetics
mitochondrial genetics Mitochondria (mitochondrion, singular) are littleorganelles found in the cytoplasm of most cells. They are
http://schools.sd68.bc.ca/ed611/phyllida/Mitochon_gen.htm

Extractions: Mitochondrial Genetics Mitochondria (mitochondrion, singular) are little organelles found in the cytoplasm of most cells. They are often referred to as the "power houses" of the cell because they are the sites of ATP (chemical energy) formation. Structually, mitochondria are bean shaped and are bounded by two membranes. The inner membrane is folded into cristae which is the site of ATP formation. Mitochondria possess their own circular (usually) DNA molecule in multiple copies. Mitochondrial DNA can be differentiated from nuclear DNA because it has a different base (examples of nitrogenous bases are adenine, thymine, cytosine and guanine) composition. Experiments have shown that the genes on mitochondrial DNA govern the normal structure and function of these organelles.
Mitochondrial Genetics Innovations And Patents
mitochondrial genetics Innovations and Patents More information on MitochondrialGenetics and mitochondrial genetics Research References.
http://www.air.xq23.com/energy_science_resources/Mitochondrial_Genetics.html
Genomic Disorders: Mutation Research, Genetic Disease
Genomic Disorders Research Centre. mitochondrial genetics.
http://www.genomic.unimelb.edu.au/pmito.html

Extractions: Workshops Related Links Site Map Dr. Ian Trounce joined the Centre in 1997 from the world's leading laboratory in this area. Numerous adult and children's disorders are caused by faults in the mitochondria and this work involves important collaborators across Melbourne. Using cell biology, genetic and biochemical approaches, we aim to improve our understanding of the variable clinical features of mitochondrial diseases to investigate mitochondrial function in neurodegenerative diseases, and create robust mtDNA mutation detection methods to define levels of somatic mtDNA mutations. Background: A major ongoing interest of the group has been to help define the cellular mechanisms underlying the extremely variable phenotypes seen in the mtDNA diseases. In particular we are interested in understanding the contributions of decreased ATP supply and increased oxidative stress, to pathology. We use cell culture methods including the transmission of mtDNAs of interest between cell lines (cybrid transfer), together with biochemical definition of key mitochondrial functions, especially oxidative phosphorylation and apoptosis. A major current project is to produce unique mouse models of mtDNA disease by introducing mtDNAs from different muridae species into mouse embryonic stem cells. In January 2001 we succeeded in producing chimeric animals from our transmitochondrial stem cells.
J. Piskur - Mitochondrial Genetics Of The Genus Saccharomyces .
93 28 09 mitochondrial genetics of the genus Saccharomyces. J. Piskur,C. Groth, M. Manuel and RF Petersen . Department of Genetics
http://genome-www.stanford.edu/Saccharomyces/yeast96/f3028.html

Extractions: Phone and Fax numbers: J. Piskur, C. Groth, M. Manuel and R.F. Petersen . Department of Genetics, University of Copenhagen, Oester Farimagsgade 2A, 1353 Copenhagen K, Denmark The Saccharomyces cerevisiae mitochondrial genome (mtDNA) consists of coding regions, introns and intergenic regions. The intergenic sequences represent two-thirds of the genome and are made up of adenine-thymine streches and numerous guanosine-cytosine (G-C) clusters. These clusters are involved in the spontaneous breakdown of mtDNA leading to petite mutants and in the transmission of mtDNA to the progeny. In the present study we examined other yeast species in the genus Saccharomyces for the organization and transmission capacities of their mtDNAs. One group of yeasts, Saccharomyces sensu stricto, have mtDNAs that are larger than 60 kilo bases (kb) and contain many G-C clusters. The other group, Saccharomyces senso lato, contains smaller mtDNAs, ranging in size from 23 to 48 kb, and has almost no G-C clusters. The lower inducibility of petites arising in the
Mitochondrial Genetics For Dummies
Yo Mama mitochondrial genetics for Dummies. Another anthropologicalmystery solved with mitochondrial genetics is that of Polynesia.
http://www.geocities.com/coligulosis/mtDNA.htm

Extractions: Yo Mama: Mitochondrial Genetics for Dummies Though I know that not everyone is as interested in stuff like this as I am, I want to provide a basic understanding of the topic for those who would like it. Plus I'm bored and a loser. That said, here is a slightly amended version of my I-Search paper (which, for those of you who don't know...was part of a semester long project all the 9th grade GATE people have to do) I know it's boring, but I think it's interesting...and you don't have to read it if you don't like. Also, take note that the paper was informal, and we had to talk about our search as well as our findings, but I ommited most of that cuz it's hella boring. And don't worry...the title of the page will be explained. What I Knew, Assumed, and Imagined What do mitochondria look like? Well...more less like this. This is a colorized electron micrograph found on the cover of the 5 March 1999 issue of Science. What I Learned My findings are varied and, I think, interesting. I discovered a lot about evolutionary studies in particular, but I also learned a lot about mitochondrial inheritance, diseases, and other aspects of mitochondrial genetics. Firstly, both chloroplasts, which serve much the same purpose in plants as mitochondria do in animals, and mitochondria were once, probably millions of years ago prokaryotic cells. These cells had an endosymbiotic relationship, a relationship in which both parties benefit, which allowed the eukaryotic cells to use sugars to create energy. Because of this, mitochondria, like bacteria, have their own set of DNA, but instead of the "x" shaped chromosomes of the nucleus, mitochondrial DNA is in the shape of a circle. The mitochondrial genome is 16,568 base pairs (bp) in length. As mitochondria produce ATP, their DNA, referred to as mtDNA, codes for the production of 13 different proteins involved in the process of producing ATP, called oxidative phosphorylation. The rest of the proteins involved in ATP production are coded for in the nucleus.
C.elegans Mitochondrial Genetics
ADP/ATP carrier protein. EC ADP/ATP transporter is the most abundant mitochondrialprotein, and is a member of the Mitochondrial transporter superfamily.
http://www.sanger.ac.uk/Users/dl1/MTDNA_2.0/TEXT/ADP_ATP_translocase.html

Extractions: This user page is hosted by the Sanger Institute EC ADP/ATP transporter is the most abundant mitochondrial protein, and is a member of the Mitochondrial transporter super-family . The protein exchanges ATP in the matrix with ADP from the exterior of the mitochondrion. The functional protein consists of a dimer embedded asymmetrically in the inner mitochondrial membrane forming a gated pore through which ADP and ATP are exchanged. In metazoan systems it has been shown that a number of isoforms of the ADT are present, (2/3 in bovine and human respectiverly). These isoforms have distinct sequences and are expressed in a tissue-specific manner, thought to be linked to the energy requirement of the tissue type. Organism Gene C.elegans [temp-#01] H.sapiens B.taurus M.musculus R.norvegicus D.melanogaster S.cerevisiae S.pombe A.thaliana Alignments : Transporters Complexes ...
Main
mitochondrial genetics (Wing 3.2). Head of Laboratory, Dr. BrigitteMeunier, PhD. Group Dr. Brigitte Meunier Group Leader, Research
http://www.ucl.ac.uk/wibr/2/research/mito.htm

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