scholarly journals Lesbian motherhood and mitochondrial replacement techniques: reproductive freedom and genetic kinship

2018 ◽  
Vol 44 (12) ◽  
pp. 835-842 ◽  
Author(s):  
Giulia Cavaliere ◽  
César Palacios-González
Keyword(s):  
Mitochondrial Dna ◽  
Therapeutic Potential ◽  
Mitochondrial Diseases ◽  
Sufficient Reason ◽  
Lesbian Couples ◽  
Reproductive Freedom ◽  
Lesbian Motherhood ◽  
Genetic Parenthood ◽  
Mitochondrial Replacement Techniques ◽  
Potential Objection

In this paper, we argue that lesbian couples who wish to have children who are genetically related to both of them should be allowed access to mitochondrial replacement techniques (MRTs). First, we provide a brief explanation of mitochondrial diseases and MRTs. We then present the reasons why MRTs are not, by nature, therapeutic. The upshot of the view that MRTs are non-therapeutic techniques is that their therapeutic potential cannot be invoked for restricting their use only to those cases where a mitochondrial DNA disease could be ‘cured’. We then argue that a positive case for MRTs is justified by an appeal to reproductive freedom, and that the criteria to access these techniques should hence be extended to include lesbian couples who wish to share genetic parenthood. Finally, we consider a potential objection to our argument: that the desire to have genetically related kin is not a morally sufficient reason to allow lesbian couples to access MRTs.

scholarly journals ‘Yes’ to mitochondrial replacement techniques and lesbian motherhood: a reply to Françoise Baylis

2018 ◽  
Vol 45 (4) ◽  
pp. 280-281
Author(s):  
César Palacios-González ◽  
Giulia Cavaliere
Keyword(s):  
Nuclear Genome ◽  
Lesbian Couples ◽  
Reproductive Freedom ◽  
Lesbian Motherhood ◽  
Mitochondrial Replacement Techniques

In a recent paper – Lesbian motherhood and mitochondrial replacement techniques: reproductive freedom and genetic kinship – we argued that lesbian couples who wish to have children who are genetically related to both of them should be allowed access to mitochondrial replacement techniques (MRTs). Françoise Baylis wrote a reply to our paper –‘No’ to lesbian motherhood using human nuclear genome transfer– where she challenges our arguments on the use of MRTs by lesbian couples, and on MRTs more generally. In this reply we respond to her claims and further clarify our position.

scholarly journals Mitochondrial DNA Replacement Techniques to Prevent Human Mitochondrial Diseases

2021 ◽  
Vol 22 (2) ◽  
pp. 551
Author(s):  
Luis Sendra ◽  
Alfredo García-Mares ◽  
María José Herrero ◽  
Salvador F. Aliño
Keyword(s):  
Mitochondrial Dna ◽  
Nuclear Dna ◽  
Genetic Disorders ◽  
Mitochondrial Diseases ◽  
Legal Regulation ◽  
Donor Oocyte ◽  
Legal Position ◽  
Ethical And Legal Issues ◽  
Mitochondrial Replacement Techniques ◽  
Do So

Background: Mitochondrial DNA (mtDNA) diseases are a group of maternally inherited genetic disorders caused by a lack of energy production. Currently, mtDNA diseases have a poor prognosis and no known cure. The chance to have unaffected offspring with a genetic link is important for the affected families, and mitochondrial replacement techniques (MRTs) allow them to do so. MRTs consist of transferring the nuclear DNA from an oocyte with pathogenic mtDNA to an enucleated donor oocyte without pathogenic mtDNA. This paper aims to determine the efficacy, associated risks, and main ethical and legal issues related to MRTs. Methods: A bibliographic review was performed on the MEDLINE and Web of Science databases, along with searches for related clinical trials and news. Results: A total of 48 publications were included for review. Five MRT procedures were identified and their efficacy was compared. Three main risks associated with MRTs were discussed, and the ethical views and legal position of MRTs were reviewed. Conclusions: MRTs are an effective approach to minimizing the risk of transmitting mtDNA diseases, but they do not remove it entirely. Global legal regulation of MRTs is required.

scholarly journals Nuclear Families: Mitochondrial Replacement Techniques and the Regulation of Parenthood

2020 ◽  
pp. 016224392093454
Author(s):  
Catherine Mills
Keyword(s):  
Mitochondrial Dna ◽  
Jacques Derrida ◽  
Nuclear Family ◽  
Nuclear Families ◽  
The United Kingdom ◽  
Egg Donors ◽  
Genetic Parenthood ◽  
Genetic Parents ◽  
The Uk ◽  
Mitochondrial Replacement Techniques

Since mitochondrial replacement techniques (MRT) were developed and clinically introduced in the United Kingdom (UK), there has been much discussion of whether these lead to children borne of three parents. In the UK, the regulation of MRT has dealt with this by stipulating that egg donors for the purposes of MRT are not genetic parents even though they contribute mitochondrial DNA (mtDNA) to offspring. In this paper, I examine the way that the Human Fertilisation and Embryology Act in the UK manages the question of parentage. I argue that the Act breaks the link typically made between genetic causation and genetic parenthood by redefining genetic causation solely in terms of nuclear genetics. Along with this, mtDNA is construed as a kind of supplement to the nuclear family. Drawing on the account of the supplement developed by Jacques Derrida, I argue that mtDNA and the women who donate it are seen as both essential to establishing the nuclear family but also exterior to and insignificant for it.

scholarly journals Does egg donation for mitochondrial replacement techniques generate parental responsibilities?

2017 ◽  
Vol 44 (12) ◽  
pp. 817-822 ◽  
Author(s):  
César Palacios-González
Keyword(s):  
Mitochondrial Dna ◽  
Nuclear Dna ◽  
Mitochondrial Diseases ◽  
Egg Donation ◽  
Genetic Composition ◽  
Numerical Identity ◽  
Egg Donor ◽  
Egg Donors ◽  
Mitochondrial Replacement Techniques

Children created through mitochondrial replacement techniques (MRTs) are commonly presented as possessing 50% of their mother’s nuclear DNA, 50% of their father’s nuclear DNA and the mitochondrial DNA of an egg donor. This lab-engineered genetic composition has prompted two questions: Do children who are the product of an MRT procedure have three genetic parents? And, do MRT egg donors have parental responsibilities for the children created? In this paper, I address the second question and in doing so I also address the first one. First, I present a brief account of mitochondrial diseases and MRTs. Second, I examine how MRTs affect the numerical identity of eggs and zygotes. Third, I investigate two genetic accounts of parenthood and MRT egg donation. Fourth, I explore three causal accounts of parenthood and MRT egg donation. My conclusion is that, under the appropriate circumstances, MRT egg donors are parentally responsible for the children created under genetic accounts of parenthood and under causal accounts of parenthood.

‘No’ to lesbian motherhood using human nuclear genome transfer

2018 ◽  
Vol 44 (12) ◽  
pp. 865-867 ◽  
Author(s):  
Françoise Baylis
Keyword(s):  
Genetic Relatedness ◽  
Reproductive Rights ◽  
Nuclear Genome ◽  
Lesbian Couples ◽  
Reproductive Freedom ◽  
Genetic Link ◽  
Lesbian Motherhood ◽  
Biological Parenthood

Giulia Cavaliere and César Palacios-González argue that lesbian couples should have access to human nuclear genome transfer (so-called mitochondrial replacement) so that both members of the couple can have a genetic link to the child they intend to parent. Their argument is grounded in an appeal to reproductive freedom. In this Response, I address a number of concerns with their argument. These concerns relate to nomenclature, treating like cases alike, genetic-relatedness and the limits of reproductive rights. On this last point, I insist that we should not mistake ‘wants’ for ‘needs’ or ‘rights’. I maintain that there is no right to biological parenthood, there is no compelling need for human nuclear genome transfer to satisfy a so-called need for genetically-related children, and we ought not to pander to an acquired desire (ie, want) for genetic filiation.

scholarly journals Clinical and molecular basis of hepatocerebral mitochondrial DNA depletion syndrome in Japan: Evaluation of outcomes after liver transplantation

2020 ◽  
Author(s):  
Masaru Shimura ◽  
Naomi Kuranobu ◽  
Minako Ogawa-Tominaga ◽  
Nana Akiyama ◽  
Yohei Sugiyama ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Mitochondrial Dna ◽  
Mitochondrial Diseases ◽  
Failure To Thrive ◽  
Neurological Complications ◽  
Mild Intellectual Disability ◽  
Initial Manifestation ◽  
Molecular Features ◽  
Mitochondrial Dna Depletion ◽  
Mitochondrial Dna Depletion Syndrome

Abstract Background Hepatocerebral mitochondrial DNA depletion syndrome (MTDPS) is a disease caused by defects in mitochondrial DNA maintenance and leads to liver failure and neurological complications during infancy. Liver transplantation (LT) remains controversial due to poor outcomes associated with extrahepatic symptoms. The purposes of this study were to clarify the current clinical and molecular features of hepatocerebral MTDPS and to evaluate the outcomes of LT in MTDPS patients in Japan.Results We retrospectively assessed the clinical and genetic findings, as well as the clinical courses, of 23 hepatocerebral MTDPS patients from a pool of 999 patients who were diagnosed with mitochondrial diseases between 2007 and 2019. Causative genes were identified in 19 of 23 patients: MPV17 (n = 13), DGUOK (n = 3), POLG (n = 1), and MICOS13 (n = 1). Eight MPV17-deficient patients harbored c.451dupC and all three DGUOK-deficient patients harbored c.143-307_170del335. The most common initial manifestation was failure to thrive (n = 13, 56.5%). The most frequent liver symptom was cholestasis (n = 21, 91.3%). LT was performed on 12 patients, including nine MPV17-deficient and two DGUOK-deficient patients. Among the 12 transplanted patients, five, including one with mild intellectual disability, survived; while seven who had remarkable neurological symptoms before LT died. Five of the MPV17-deficient survivors had either c.149G>A or c.293C>T. Conclusions MPV17 was the most common genetic cause of hepatocerebral MTDPS. The outcome of LT for MTDPS was not favorable, as previously reported, however, patients harboring MPV17 mutations associated with mild phenotypes such as c.149G>A or c.293C>T, and exhibiting no marked neurologic manifestations before LT, had a better prognosis after LT.

33. Therapeutic potential of pyruvate for mitochondrial diseases

Mitochondrion ◽  
2009 ◽  
Vol 9 (1) ◽  
pp. 69
Author(s):  
Masashi Tanaka ◽  
Yutaka Nishigaki ◽  
Noriyuki Fuku ◽  
Tohru Ibi ◽  
Ko Sahashi ◽  
...  
Keyword(s):  
Therapeutic Potential ◽  
Mitochondrial Diseases

scholarly journals DRP1 regulates endoplasmic reticulum sheets to control mitochondrial DNA replication and segregation

2021 ◽  
Author(s):  
Hema Saranya Ilamathi ◽  
Sara Benhammouda ◽  
Justine Desrochers-Goyette ◽  
Matthew A Lines ◽  
Marc Germain
Keyword(s):  
Endoplasmic Reticulum ◽  
Mitochondrial Dna ◽  
Protein Complexes ◽  
Mitochondrial Dynamics ◽  
Mitochondrial Fission ◽  
Mitochondrial Diseases ◽  
Contact Sites ◽  
Transport Chain ◽  
Essential Components ◽  
Mtdna Replication

Mitochondria are multi-faceted organelles crucial for cellular homeostasis that contain their own genome. Mitochondrial DNA (mtDNA) codes for several essential components of the electron transport chain, and mtDNA maintenance defects lead to mitochondrial diseases. mtDNA replication occurs at endoplasmic reticulum (ER)-mitochondria contact sites and is regulated by mitochondrial dynamics. Specifically, mitochondrial fusion is essential for mtDNA maintenance. In contrast, while loss of mitochondrial fission causes the aggregation of nucleoids (mtDNA-protein complexes), its role in nucleoid distribution remains unclear. Here, we show that the mitochondrial fission protein DRP1 regulates nucleoid segregation by altering ER sheets, the ER structure associated with protein synthesis. Specifically, DRP1 loss or mutation leads to altered ER sheets that physically interact with mitobulbs, mitochondrial structures containing aggregated nucleoids. Importantly, nucleoid distribution and mtDNA replication were rescued by expressing the ER sheet protein CLIMP63. Thus, our work identifies a novel mechanism by which DRP1 regulates mtDNA replication and distribution.

scholarly journals Segregation of mitochondrial DNA (mtDNA) in human oocytes and in animal models of mtDNA disease: clinical implications

Reproduction ◽  
2002 ◽  
pp. 751-755 ◽  
Author(s):  
J Poulton ◽  
DR Marchington
Keyword(s):  
Mitochondrial Dna ◽  
Antenatal Diagnosis ◽  
Mitochondrial Diseases ◽  
Management Options ◽  
Mtdna Mutations ◽  
Mendelian Disorders ◽  
Leigh’S Syndrome ◽  
Normal Individuals ◽  
Substantial Progress ◽  
Chorionic Villous Sampling

Mitochondrial DNA (mtDNA) is almost entirely maternally inherited. Thousands of copies of mtDNA are present in every nucleated cell and in most normal individuals these are virtually identical (homoplasmy). mtDNA diseases may be caused by mutations in either mitochondrial or nuclear genes and, hence, give rise to maternal or autosomal patterns of inheritance. Antenatal diagnosis of mitochondrial diseases based on chorionic villous sampling is available for Mendelian disorders and the syndromes caused by mutations at bp 8993 (associated with Leigh's syndrome and neurogenic weakness, ataxia and retinitis pigmentosa (NARP)). However, prenatal diagnosis of many other maternally inherited mtDNA diseases is less reliable because it is not possible to predict with confidence the way in which heteroplasmic mtDNA mutations segregate within tissues and find clinical expression. This review focuses on the substantial progress in genetics that has been made recently, and on the management options that clinicians can offer to families.

Sign in / Sign up

Export Citation Format

Share Document