652 Ergebnisse für: "Sulfuric Acid"

Supplier: Bioss

Belonging to the CAF17 subfamily of the gcvT family, C1orf69 is a 356 amino acid mitochondrial protein that is required for normal heme synthesis. Heme synthesis is dependent upon receiving iron through iron sulfur (Fe-S) cluster biogenesis proteins such as C1orf69, which is specifically thought to assemble Fe-S clusters for mitochondrial aconitase and lipoate synthase. In zebrafish, deficiency of C1orf69 causes anemia. The gene encoding C1orf69 maps to human chromosome 1, the largest human chromosome spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, and considering the great number of genes there are also a large number of diseases associated with chromosome 1.

Supplier: Bioss

Belonging to the CAF17 subfamily of the gcvT family, C1orf69 is a 356 amino acid mitochondrial protein that is required for normal heme synthesis. Heme synthesis is dependent upon receiving iron through iron sulfur (Fe-S) cluster biogenesis proteins such as C1orf69, which is specifically thought to assemble Fe-S clusters for mitochondrial aconitase and lipoate synthase. In zebrafish, deficiency of C1orf69 causes anemia. The gene encoding C1orf69 maps to human chromosome 1, the largest human chromosome spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, and considering the great number of genes there are also a large number of diseases associated with chromosome 1.

Supplier: Bioss

Belonging to the CAF17 subfamily of the gcvT family, C1orf69 is a 356 amino acid mitochondrial protein that is required for normal heme synthesis. Heme synthesis is dependent upon receiving iron through iron sulfur (Fe-S) cluster biogenesis proteins such as C1orf69, which is specifically thought to assemble Fe-S clusters for mitochondrial aconitase and lipoate synthase. In zebrafish, deficiency of C1orf69 causes anemia. The gene encoding C1orf69 maps to human chromosome 1, the largest human chromosome spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, and considering the great number of genes there are also a large number of diseases associated with chromosome 1.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Belonging to the CAF17 subfamily of the gcvT family, C1orf69 is a 356 amino acid mitochondrial protein that is required for normal heme synthesis. Heme synthesis is dependent upon receiving iron through iron sulfur (Fe-S) cluster biogenesis proteins such as C1orf69, which is specifically thought to assemble Fe-S clusters for mitochondrial aconitase and lipoate synthase. In zebrafish, deficiency of C1orf69 causes anemia. The gene encoding C1orf69 maps to human chromosome 1, the largest human chromosome spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, and considering the great number of genes there are also a large number of diseases associated with chromosome 1.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Spectrum Chemical

Sulfuric Acid, FCC is considered a general purpose food additive and is used to directly control pH during the processing of food by discouraging the growth of bacteria and spoilage microbes. The FCC grade meets the requirements of the Food Chemical Codex indicates and is suitable for all food, beverage and nutritional supplement applications. Spectrum Chemical offers over 300 Food grade chemical ingredients packaged in laboratory size bottles to production drum quantities and are manufactured, packaged and stored under current Good Manufacturing Practices (cGMP) per 21CFR part 211 in FDA registered and inspected facilities.

Supplier: Bioss

Mitoferrin 2, also known as MRS3/4 (mitochondrial RNA-splicing protein 3/4 homolog), mitochondrial iron transporter 2, NPD016, MRS4L or SLC25A28 (solute carrier family 25 member 28), is a 364 amino acid multi-pass membrane protein of the mitochondrial inner membrane that mediates iron uptake. Mitoferrin 2 is thought to play a role in heme synthesis of hemoproteins and iron-sulfur cluster assembly. Ubiquitously expressed, Mitoferrin 2 is found at high levels in skeletal muscle, heart, placenta, kidney, lung, liver, brain and pancreas. Mitoferrin 2 is a member of the mitochondrial carrier family and undergoes alternative splicing events to produce four isoforms. Mitoferrin 2 contains three solcar repeats and is encoded by a gene that maps to human chromosome 10q24.2.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidising NADH that is produced in the Krebs cycle, this complex utilises the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest Zince defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterised by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterised by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Supplier: Bioss

Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.