Difference between revisions of "Iyer 2008 Nature Precedings"
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{{Publication | {{Publication | ||
|title=Iyer S, Thomas R, Portell F, Dunham L, Quigley C, Bennett JP (2008) Recombinant mitochondrial transcription factor A with N-terminal mitochondrial transduction domain increases respiration and mitochondrial gene expression in G11778A leber’s hereditary optic neuropathy cybrid cells. Nature Precedings : hdl:10101/npre.2008.2084.1 | |title=Iyer S, Thomas R, Portell F, Dunham L, Quigley C, Bennett JP (2008) Recombinant mitochondrial transcription factor A with N-terminal mitochondrial transduction domain increases respiration and mitochondrial gene expression in G11778A leber’s hereditary optic neuropathy cybrid cells. Nature Precedings: hdl:10101/npre.2008.2084.1 | ||
|info=[http://precedings.nature.com/documents/2084/version/1/files/npre20082084-1.pdf Nature Precedings : hdl:10101/npre.2008.2084.1] | |info=[http://precedings.nature.com/documents/2084/version/1/files/npre20082084-1.pdf Nature Precedings : hdl:10101/npre.2008.2084.1] | ||
|authors=Iyer | |authors=Iyer Shilpa, Thomas R, Portell F, Dunham L, Quigley C, Bennett JP | ||
|year=2008 | |year=2008 | ||
|journal=Nature | |journal=Nature Precedings | ||
|abstract=Diseases involving mitochondrial defects usually manifest themselves in highenergy, post-mitotic tissues such as brain, retina, skeletal and cardiac muscle and frequently cause deficiencies in mitochondrial bioenergetics1, 2. We have developed a scalable procedure to produce recombinant human mitochondrial transcription factor A (TFAM) 3-5 modified with an N-terminal protein transduction domain (PTD)6 and mitochondrial localization signal (MLS) that allow it to cross membranes and enter mitochondria through its “mitochondrial transduction domain” (MTD,=PTD+MLS). In vitro studies in a classic mitochondrial disease cell model demonstrated that Alexa488-labeled MTD-TFAM rapidly entered the mitochondrial compartment. MTD-TFAM treatment of these cell lines reversibly increased oxygen consumption (respiration) rates 3-fold, levels of respiratory proteins and mitochondrial gene expression. In vivo results demonstrated that respiration increased to lesser degrees in mitochondria from tissues of mice injected with MTD-TFAM. MTD-TFAM can alter mitochondrial bioenergetics and holds promise for treatment of mitochondrial diseases involving deficiencies of energy production. | |abstract=Diseases involving mitochondrial defects usually manifest themselves in highenergy, post-mitotic tissues such as brain, retina, skeletal and cardiac muscle and frequently cause deficiencies in mitochondrial bioenergetics1, 2. We have developed a scalable procedure to produce recombinant human mitochondrial transcription factor A (TFAM) 3-5 modified with an N-terminal protein transduction domain (PTD)6 and mitochondrial localization signal (MLS) that allow it to cross membranes and enter mitochondria through its “mitochondrial transduction domain” (MTD,=PTD+MLS). In vitro studies in a classic mitochondrial disease cell model demonstrated that Alexa488-labeled MTD-TFAM rapidly entered the mitochondrial compartment. MTD-TFAM treatment of these cell lines reversibly increased oxygen consumption (respiration) rates 3-fold, levels of respiratory proteins and mitochondrial gene expression. In vivo results demonstrated that respiration increased to lesser degrees in mitochondria from tissues of mice injected with MTD-TFAM. MTD-TFAM can alter mitochondrial bioenergetics and holds promise for treatment of mitochondrial diseases involving deficiencies of energy production. | ||
|keywords=TFAM, Respiration, Mitochondrial gene expression, Respiratory proteins | |keywords=TFAM, Respiration, Mitochondrial gene expression, Respiratory proteins | ||
|mipnetlab=US_VA Richmond_Bennett JP | |mipnetlab=US_VA Richmond_Bennett JP, US VA Richmond Iyer S | ||
|discipline=Mitochondrial Physiology, Biomedicine | |discipline=Mitochondrial Physiology, Biomedicine | ||
}} | }} | ||
{{Labeling | {{Labeling | ||
| | |area=Respiration, Genetic knockout;overexpression, mt-Medicine | ||
| | |tissues=Nervous system | ||
| | |diseases=Inherited | ||
| | |couplingstates=OXPHOS | ||
|instruments=Oxygraph-2k | |||
|discipline=Mitochondrial Physiology, Biomedicine | |discipline=Mitochondrial Physiology, Biomedicine | ||
}} | }} | ||
Latest revision as of 16:33, 6 May 2020
| Iyer S, Thomas R, Portell F, Dunham L, Quigley C, Bennett JP (2008) Recombinant mitochondrial transcription factor A with N-terminal mitochondrial transduction domain increases respiration and mitochondrial gene expression in G11778A leber’s hereditary optic neuropathy cybrid cells. Nature Precedings: hdl:10101/npre.2008.2084.1 |
» Nature Precedings : hdl:10101/npre.2008.2084.1
Iyer Shilpa, Thomas R, Portell F, Dunham L, Quigley C, Bennett JP (2008) Nature Precedings
Abstract: Diseases involving mitochondrial defects usually manifest themselves in highenergy, post-mitotic tissues such as brain, retina, skeletal and cardiac muscle and frequently cause deficiencies in mitochondrial bioenergetics1, 2. We have developed a scalable procedure to produce recombinant human mitochondrial transcription factor A (TFAM) 3-5 modified with an N-terminal protein transduction domain (PTD)6 and mitochondrial localization signal (MLS) that allow it to cross membranes and enter mitochondria through its “mitochondrial transduction domain” (MTD,=PTD+MLS). In vitro studies in a classic mitochondrial disease cell model demonstrated that Alexa488-labeled MTD-TFAM rapidly entered the mitochondrial compartment. MTD-TFAM treatment of these cell lines reversibly increased oxygen consumption (respiration) rates 3-fold, levels of respiratory proteins and mitochondrial gene expression. In vivo results demonstrated that respiration increased to lesser degrees in mitochondria from tissues of mice injected with MTD-TFAM. MTD-TFAM can alter mitochondrial bioenergetics and holds promise for treatment of mitochondrial diseases involving deficiencies of energy production. • Keywords: TFAM, Respiration, Mitochondrial gene expression, Respiratory proteins
• O2k-Network Lab: US_VA Richmond_Bennett JP, US VA Richmond Iyer S
Labels: MiParea: Respiration, Genetic knockout;overexpression, mt-Medicine
Pathology: Inherited
Tissue;cell: Nervous system
Coupling state: OXPHOS
HRR: Oxygraph-2k
