Human mitochondrial DNA with the 37 genes on their respective
H- and L-strands. Electron microscopy reveals mitochondrial DNA
in discrete foci. Bars: 200 nm. (A) Cytoplasmic section after
immunogold labelling with anti-DNA; gold particles marking mt
found near the mitochondrial membrane. (B) Whole mount view of
cytoplasm after extraction with CSK buffer and immunogold labelling
with anti-DNA; mt
DNA (marked by gold particles) resists extraction.
From Iborra et al., 2004.
DNA or MDNA) is the
DNA located in mitochondria
, cellular organelles within eukaryotic cells that convert chemical
energy from food into a form that cells can use, adenosine
triphosphate (ATP). Mitochondrial
DNA is only a small portion of the
DNA in a eukaryotic cell; most of the
DNA can be found in the cell
nucleus and, in plants and algae, also in plastids such as
In humans, the 16,569 base pairs of mitochondrial
DNA encode for only
37 genes .
Human mitochondrial DNA was the first significant part of
the human genome to be sequenced. In most species, including humans,
DNA is inherited solely from the mother.
Since animal mt
DNA evolves faster than nuclear genetic markers, it
represents a mainstay of phylogenetics and evolutionary biology. It
also permits an examination of the relatedness of populations, and so
has become important in anthropology and biogeography .
* 1 Origin
* 2 Mitochondrial inheritance
* 2.1 Female inheritance
* 2.2 The mitochondrial bottleneck
* 2.3 Male inheritance
* 3 Structure
* 3.1 Circular versus linear
* 3.2 In mammals
* 3.3 In plants
* 3.4 In protists
* 4 Genome diversity
* 4.1 Animals
* 4.2 Plants and fungi
* 4.3 Protists
* 5 Replication
* 6 Transcription
* 7 Mutations and disease
* 7.1 Susceptibility
* 7.2 Genetic illness
* 7.3 Use in disease diagnosis
* 7.4 Relationship with aging
* 7.5 Correlation of the mt
DNA base composition with animals
* 7.6 Relationship with non-B (non-canonical)
* 8 Use in identification
* 9 History
* 10 Mitochondrial sequence databases
* 11 Mitochondrial mutation databases
* 12 See also
* 13 References
Nuclear and mitochondrial
DNA are thought to be of separate
evolutionary origin, with the mt
DNA being derived from the circular
genomes of the bacteria that were engulfed by the early ancestors of
today's eukaryotic cells. This theory is called the endosymbiotic
theory . Each mitochondrion is estimated to contain 2–10 mtDNA
copies. In the cells of extant organisms, the vast majority of the
proteins present in the mitochondria (numbering approximately 1500
different types in mammals ) are coded for by nuclear
DNA , but the
genes for some of them, if not most, are thought to have originally
been of bacterial origin, having since been transferred to the
eukaryotic nucleus during evolution .
The reasons why mitochondria have retained some genes are debated.
The existence in some species of mitochondrion-derived organelles
lacking a genome suggests that complete gene loss is possible, and
transferring mitochondrial genes to the nucleus has several
advantages. The difficulty of targeting remotely-produced hydrophobic
protein products to the mitochondrion is one hypothesis for why some
genes are retained in mtDNA; colocalisation for redox regulation is
another, citing the desirability of localised control over
mitochondrial machinery. Recent analysis of a wide range of mtDNA
genomes suggests that both these features may dictate mitochondrial
In most multicellular organisms , mt
DNA is inherited from the mother
(maternally inherited). Mechanisms for this include simple dilution
(an egg contains on average 200,000 mt
DNA molecules, whereas a healthy
human sperm was reported to contain on average 5 molecules ),
degradation of sperm mt
DNA in the male genital tract, in the
fertilized egg, and, at least in a few organisms, failure of sperm
DNA to enter the egg. Whatever the mechanism, this single parent
(uniparental inheritance ) pattern of mt
DNA inheritance is found in
most animals, most plants and in fungi as well.
In sexual reproduction , mitochondria are normally inherited
exclusively from the mother; the mitochondria in mammalian sperm are
usually destroyed by the egg cell after fertilization. Also, most
mitochondria are present at the base of the sperm's tail, which is
used for propelling the sperm cells; sometimes the tail is lost during
fertilization. In 1999 it was reported that paternal sperm
mitochondria (containing mtDNA) are marked with ubiquitin to select
them for later destruction inside the embryo . Some in vitro
fertilization techniques, particularly injecting a sperm into an
oocyte , may interfere with this.
The fact that mitochondrial
DNA is maternally inherited enables
genealogical researchers to trace maternal lineage far back in time.
DNA , paternally inherited, is used in an analogous way
to determine the patrilineal history.) This is usually accomplished on
DNA by sequencing the hypervariable control
regions (HVR1 or HVR2), and sometimes the complete molecule of the
mitochondrial DNA, as a genealogical
DNA test . HVR1, for example,
consists of about 440 base pairs. These 440 base pairs are then
compared to the control regions of other individuals (either specific
people or subjects in a database) to determine maternal lineage. Most
often, the comparison is made to the revised Cambridge Reference
Sequence . Vilà et al. have published studies tracing the matrilineal
descent of domestic dogs to wolves. The concept of the Mitochondrial
Eve is based on the same type of analysis, attempting to discover the
origin of humanity by tracking the lineage back in time.
DNA is highly conserved, and its relatively slow mutation rates
(compared to other
DNA regions such as microsatellites ) make it
useful for studying the evolutionary relationships—phylogeny —of
organisms. Biologists can determine and then compare mt
among different species and use the comparisons to build an
evolutionary tree for the species examined. However, due to the slow
mutation rates it experiences, it is often hard to distinguish between
closely related species to any large degree, so other methods of
analysis must be used.
THE MITOCHONDRIAL BOTTLENECK
Entities undergoing uniparental inheritance and with little to no
recombination may be expected to be subject to Muller\'s ratchet , the
accumulation of deleterious mutations until functionality is lost.
Animal populations of mitochondria avoid this buildup through a
developmental process known as the mt
DNA bottleneck . The bottleneck
exploits stochastic processes in the cell to increase in the
cell-to-cell variability in mutant load as an organism develops: a
single egg cell with some proportion of mutant mt
DNA thus produces an
embryo where different cells have different mutant loads. Cell-level
selection may then act to remove those cells with more mutant mtDNA,
leading to a stabilisation or reduction in mutant load between
generations. The mechanism underlying the bottleneck is debated,
with a recent mathematical and experimental metastudy providing
evidence for a combination of random partitioning of mtDNAs at cell
divisions and random turnover of mt
DNA molecules within the cell.
Main article: Paternal mt
Doubly uniparental inheritance of mt
DNA is observed in bivalve
mollusks. In those species, females have only one type of mt
whereas males have F type mt
DNA in their somatic cells, but M type of
DNA (which can be as much as 30% divergent) in germline cells.
Paternally inherited mitochondria have additionally been reported in
some insects such as fruit flies , honeybees , and periodical
Male mitochondrial inheritance was recently discovered in Plymouth
Rock chickens . Evidence supports rare instances of male
mitochondrial inheritance in some mammals as well. Specifically,
documented occurrences exist for mice, where the male-inherited
mitochondria were subsequently rejected. It has also been found in
sheep, and in cloned cattle. It has been found in a single case in a
Although many of these cases involve cloned embryos or subsequent
rejection of the paternal mitochondria, others document in vivo
inheritance and persistence under lab conditions.
An IVF technique known as mitochondrial donation or mitochondrial
replacement therapy (MRT) results in offspring containing mt
DNA from a
donor female, and nuclear
DNA from the mother and father. In the
spindle transfer procedure, the nucleus of an egg is inserted into the
cytoplasm of an egg from a donor female which has had its nucleus
removed, but still contains the donor female's mtDNA. The composite
egg is then fertilized with the male's sperm. The procedure is used
when a woman with genetically defective mitochondria wishes to
procreate and produce offspring with healthy mitochondria. The first
known child to be born as a result of mitochondrial donation was a boy
born to a Jordanian couple in Mexico on 6 April 2016.
CIRCULAR VERSUS LINEAR
In most multicellular organisms, the mt
DNA - or mitogenome - is
organized as a circular, covalently closed, double-stranded
DNA . But
in many unicellular (e.g. the ciliate
Tetrahymena or the green alga
Chlamydomonas reinhardtii ) and in rare cases also in multicellular
organisms (e.g. in some species of
Cnidaria ) the mt
DNA is found as
DNA . Most of these linear mtDNAs possess
telomerase independent telomeres (i.e. the ends of the linear
with different modes of replication, which have made them interesting
objects of research, as many of these unicellular organisms with
DNA are known pathogens .
For human mitochondrial
DNA (and probably for that of metazoans in
general), 100-10,000 separate copies of mt
DNA are usually present per
somatic cell (egg and sperm cells are exceptions). In mammals, each
double-stranded circular mt
DNA molecule consists of 15,000-17,000
base pairs . The two strands of mt
DNA are differentiated by their
nucleotide content, with a guanine -rich strand referred to as the
heavy strand (or H-strand) and a cytosine -rich strand referred to as
the light strand (or L-strand). The heavy strand encodes 28 genes, and
the light strand encodes 9 genes for a total of 37 genes. Of the 37
genes, 13 are for proteins (polypeptides), 22 are for transfer RNA
(tRNA) and two are for the small and large subunits of ribosomal RNA
(rRNA). The human mitogenome contains overlapping genes (ATP8 and
ATP6 as well as ND4L and ND4 : see the human mitochondrial genome map
), a feature that is rare in animal genomes. The 37-gene pattern is
also seen among most metazoans, although in some cases one or more of
these genes is absent and the mt
DNA size range is greater.
The 37 genes of the
Cambridge Reference Sequence for human
DNA and their locations
in the mitogenome STRAND
ATP synthase , Fo subunit 8 (complex V)
08,366-08,572 (overlap with MT-ATP6)
ATP synthase , Fo subunit 6 (complex V)
08,527-09,207 (overlap with MT-ATP8)
Cytochrome c oxidase , subunit 1 (complex IV)
Cytochrome c oxidase , subunit 2 (complex IV)
Cytochrome c oxidase , subunit 3 (complex IV)
Cytochrome b (complex III)
NADH dehydrogenase , subunit 1 (complex I)
NADH dehydrogenase , subunit 2 (complex I)
NADH dehydrogenase , subunit 3 (complex I)
NADH dehydrogenase , subunit 4L (complex I)
NADH dehydrogenase , subunit 4 (complex I)
10,760-12,137 (overlap with MT-ND4L)
NADH dehydrogenase , subunit 5 (complex I)
NADH dehydrogenase , subunit 6 (complex I)
Alanine (Ala or A)
Arginine (Arg or R)
Asparagine (Asn or N)
Aspartic acid (Asp or D)
Cysteine (Cys or C)
Glutamic acid (Glu or E)
Glutamine (Gln or Q)
Glycine (Gly or G)
Histidine (His or H)
Isoleucine (Ile or I)
Leucine (Leu-UUR or L)
Leucine (Leu-CUN or L)
Lysine (Lys or K)
Methionine (Met or M)
Phenylalanine (Phe or F)
Proline (Pro or P)
Serine (Ser-UCN or S)
Serine (Ser-AGY or S)
Threonine (Thr or T)
Tryptophan (Trp or W)
Tyrosine (Tyr or Y)
Valine (Val or V)
Small subunit : SSU (12S)
Large subunit : LSU (16S)
Great variation in mt
DNA gene content and size exists among fungi and
plants, although there appears to be a core subset of genes that are
present in all eukaryotes (except for the few that have no
mitochondria at all). Some plant species have enormous mitochondrial
genomes, with Silene conica mt
DNA containing as many as 11,300,000
base pairs. Surprisingly, even those huge mtDNAs contain the same
number and kinds of genes as related plants with much smaller mtDNAs.
The genome of the mitochondrion of the cucumber (
Cucumis sativus )
consists of three circular chromosomes (lengths 1556, 84 and 45
kilobases), which are entirely or largely autonomous with regard to
their replication .
The smallest mitochondrial genome sequenced to date is the 5967 bp
DNA of the parasite
Plasmodium falciparum .
There are six main genome types found in mitochondrial genomes. These
genome types were classified by “Kolesnikov "> Human
DNA with groups of protein-, rRNA- and tRNA-encoding
genes. The involvement of mitochondrial
DNA in several human
The concept that mt
DNA is particularly susceptible to reactive oxygen
species generated by the respiratory chain due to its proximity
remains controversial. mt
DNA does not accumulate any more oxidative
base damage than nuclear DNA. It has been reported that at least some
types of oxidative
DNA damage are repaired more efficiently in
mitochondria than they are in the nucleus. mt
DNA is packaged with
proteins which appear to be as protective as proteins of the nuclear
chromatin. Moreover, mitochondria evolved a unique mechanism which
DNA integrity through degradation of excessively damaged
genomes followed by replication of intact/repaired mtDNA. This
mechanism is not present in the nucleus and is enabled by multiple
copies of mt
DNA present in mitochondria The outcome of mutation in
DNA may be an alteration in the coding instructions for some
proteins, which may have an effect on organism metabolism and/or
Mutations of mitochondrial
DNA can lead to a number of illnesses
including exercise intolerance and
Kearns–Sayre syndrome (KSS),
which causes a person to lose full function of heart, eye, and muscle
movements. Some evidence suggests that they might be major
contributors to the aging process and age-associated pathologies .
Particularly in the context of disease, the proportion of mutant mtDNA
molecules in a cell is termed heteroplasmy . The within-cell and
between-cell distributions of heteroplasmy dictate the onset and
severity of disease and are influenced by complicated stochastic
processes within the cell and during development.
Mutations in mitochondrial tRNAs can be responsible for severe
diseases like the MELAS and MERRF syndromes.
Mutations in nuclear genes that encode proteins that mitochondria use
can also contribute to mitochondrial diseases. These diseases do not
follow mitochondrial inheritance patterns, but instead follow
Mendelian inheritance patterns.
USE IN DISEASE DIAGNOSIS
Recently a mutation in mt
DNA has been used to help diagnose prostate
cancer in patients with negative prostate biopsy .
RELATIONSHIP WITH AGING
Though the idea is controversial, some evidence suggests a link
between aging and mitochondrial genome dysfunction. In essence,
mutations in mt
DNA upset a careful balance of reactive oxygen species
(ROS) production and enzymatic ROS scavenging (by enzymes like
superoxide dismutase , catalase , glutathione peroxidase and others).
However, some mutations that increase ROS production (e.g., by
reducing antioxidant defenses) in worms increase, rather than
decrease, their longevity. Also, naked mole rats , rodents about the
size of mice , live about eight times longer than mice despite having
reduced, compared to mice, antioxidant defenses and increased
oxidative damage to biomolecules. Once, there was thought to be a
positive feedback loop at work (a 'Vicious Cycle'); as mitochondrial
DNA accumulates genetic damage caused by free radicals, the
mitochondria lose function and leak free radicals into the cytosol. A
decrease in mitochondrial function reduces overall metabolic
efficiency. However, this concept was conclusively disproved when it
was demonstrated that mice, which were genetically altered to
DNA mutations at accelerated rate do age prematurely, but
their tissues do not produce more ROS as predicted by the 'Vicious
Cycle' hypothesis. Supporting a link between longevity and
mitochondrial DNA, some studies have found correlations between
biochemical properties of the mitochondrial
DNA and the longevity of
species. Extensive research is being conducted to further investigate
this link and methods to combat aging. Presently, gene therapy and
nutraceutical supplementation are popular areas of ongoing research.
Bjelakovic et al. analyzed the results of 78 studies between 1977 and
2012, involving a total of 296,707 participants, and concluded that
antioxidant supplements do not reduce all-cause mortality nor extend
lifespan, while some of them, such as beta carotene, vitamin E, and
higher doses of vitamin A, may actually increase mortality.
CORRELATION OF THE MT
DNA BASE COMPOSITION WITH ANIMALS LIFESPAN
Animal species mt
DNA base composition was retrieved from the
MitoAge database and compared to their maximum life span from AnAge
Over the past decade, an Israeli research group led by Professor
Vadim Fraifeld has shown that extraordinarily strong and significant
correlations exist between the mt
DNA base composition and animal
species-specific maximum life spans. As demonstrated in their work,
DNA guanine + cytosine content (GC% ) strongly associates
with longer maximum life spans across animal species. An additional
astonishing observation is that the mt
DNA GC% correlation with the
maximum life spans is independent of the well-known correlation
between animal species metabolic rate and maximum life spans. The
DNA GC% and resting metabolic rate explain the differences in animal
species maximum life spans in a multiplicative manner (i.e., species
maximum life span = their mt
DNA GC% * metabolic rate). To support the
scientific community in carrying out comparative analyses between
DNA features and longevity across animals, a dedicated database was
built named MitoAge.
RELATIONSHIP WITH NON-B (NON-CANONICAL)
Deletion breakpoints frequently occur within or near regions showing
non-canonical (non-B) conformations, namely hairpins, cruciforms and
cloverleaf-like elements. Moreover, there is data supporting the
involvement of helix-distorting intrinsically curved regions and long
G-tetrads in eliciting instability events. In addition, higher
breakpoint densities were consistently observed within GC-skewed
regions and in the close vicinity of the degenerate sequence motif
USE IN IDENTIFICATION
For use in human identification, see
Human mitochondrial DNA .
Unlike nuclear DNA, which is inherited from both parents and in which
genes are rearranged in the process of recombination , there is
usually no change in mt
DNA from parent to offspring. Although mtDNA
also recombines, it does so with copies of itself within the same
mitochondrion. Because of this and because the mutation rate of animal
DNA is higher than that of nuclear DNA, mt
DNA is a powerful tool
for tracking ancestry through females (matrilineage ) and has been
used in this role to track the ancestry of many species back hundreds
The rapid mutation rate (in animals) makes mt
DNA useful for assessing
genetic relationships of individuals or groups within a species and
also for identifying and quantifying the phylogeny (evolutionary
relationships; see phylogenetics ) among different species. To do
this, biologists determine and then compare the mt
DNA sequences from
different individuals or species. Data from the comparisons is used to
construct a network of relationships among the sequences, which
provides an estimate of the relationships among the individuals or
species from which the mtDNAs were taken. mt
DNA can be used to
estimate the relationship between both closely related and distantly
related species. Due to the high mutation rate of mt
DNA in animals,
the 3rd positions of the codons change relatively rapidly, and thus
provide information about the genetic distances among closely related
individuals or species. On the other hand, the substitution rate of
mt-proteins is very low, thus amino acid changes accumulate slowly
(with corresponding slow changes at 1st and 2nd codon positions) and
thus they provide information about the genetic distances of distantly
related species. Statistical models that treat substitution rates
among codon positions separately, can thus be used to simultaneously
estimate phylogenies that contain both closely and distantly related
DNA was admitted into evidence for the first time ever
in 1996 during State of Tennessee v. Paul Ware.
In the 1998 court case of Commonwealth of Pennsylvania v. Patricia
Lynne Rorrer, mitochondrial
DNA was admitted into evidence in the
State of Pennsylvania for the first time. The case was featured in
episode 55 of season 5 of the true crime drama series Forensic Files
(season 5) .
DNA was first admitted into evidence in
the successful prosecution of David Westerfield for the 2002
kidnapping and murder of 7-year-old Danielle van Dam in
San Diego : it
was used for both human and dog identification. This was the first
trial in the U.S. to admit canine DNA.
The remains of King Richard III were identified by comparing his
DNA with that of two matrilineal descendants of his sister.
DNA was discovered in the 1960s by Margit M. K. Nass
and Sylvan Nass by electron microscopy as DNase-sensitive threads
inside mitochondria, and by Ellen Haslbrunner,
Hans Tuppy and
Gottfried Schatz by biochemical assays on highly purified
MITOCHONDRIAL SEQUENCE DATABASES
Several specialized databases have been founded to collect
mitochondrial genome sequences and other information. Although most of
them focus on sequence data, some of them include phylogenetic or
* MITOSATPLANT: Mitochondrial microsatellites database of
* MITOBREAK: the mitochondrial
DNA breakpoints database.
* MITOFISH and MITOANNOTATOR: a mitochondrial genome database of
fish. See also Cawthorn et al.
* MITOZOA 2.0: a database for comparative and evolutionary analyses
of mitochondrial genomes in Metazoa. (no longer available)
* INTERMITOBASE: an annotated database and analysis platform of
protein-protein interactions for human mitochondria. (apparently last
updated in 2010, but still available)
* MITOME: a database for comparative mitochondrial genomics in
metazoan animals (no longer available)
* MITORES: a resource of nuclear-encoded mitochondrial genes and
their products in metazoa (apparently no longer being updated)
MITOCHONDRIAL MUTATION DATABASES
Several specialized databases exist that report polymorphisms and
mutations in the human mitochondrial DNA, together with the assessment
of their pathogenicity.
* MITOMAP: A compendium of polymorphisms and mutations in human
* MITIMPACT: A collection of pre-computed pathogenicity predictions
for all nucleotide changes that cause non-synonymous substitutions in
human mitochondrial protein coding genes .
Wikimedia Commons has media related to MITOCHONDRIAL
Archaeogenetics of the Near East
Human mitochondrial DNA haplogroup
Human mitochondrial genetics
* Mitochondrial rCRS
* Paternal mt
Single origin theory
Genetic history of Africa
Genetic history of Europe
Genetic history of the British Isles
Genetic history of the Iberian Peninsula
Genetic history of indigenous peoples of the Americas
Genetic history of Italy
Genetic history of North Africa
Genetic history of North Africa
Genetics and archaeogenetics of South Asia
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Human mitochondrial DNA
Types of nucleic acids
(coding, non-coding )
* precursor, heterogenous nuclear
* Small interfering
* Small nuclear
* Small nucleolar
* Small Cajal Body RNAs
* Small hairpin
* Small temporal
* Trans-acting small interfering
* Subgenomic messenger
* Multicopy single-stranded
* Artificial chromosomes
FATTY ACID DEGRADATION
Carnitine palmitoyltransferase I
Coenzyme Q – cytochrome c reductase
Glycerol phosphate shuttle
Glutamate aspartate transporter
Carnitine palmitoyltransferase II
CITRIC ACID CYCLE
Oxoglutarate dehydrogenase complex
Succinyl coenzyme A synthetase
Pyruvate dehydrogenase complex
Carbamoyl phosphate synthetase I
OTHER/TO BE SORTED
Cholesterol side-chain cleavage enzyme
Mitochondrial membrane transport protein
Mitochondrial permeability transition pore
see also mitochondrial diseases
* GND : 4202301-4
* Molecular and cellular biology portal