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UQCRC1 | ubiquinol-cytochrome c reductase core protein I; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This protein may mediate formation of the complex between cytochromes c and c1 (480 aa) | |||
ATP6V1D | ATPase, H+ transporting, lysosomal 34kDa, V1 subunit D; Subunit of the peripheral V1 complex of vacuolar ATPase. Vacuolar ATPase is responsible for acidifying a variety of intracellular compartments in eukaryotic cells, thus providing most of the energy required for transport processes in the vacuolar system (By similarity) (247 aa) | |||
GCLC | glutamate-cysteine ligase, catalytic subunit (637 aa) | |||
NARFL | nuclear prelamin A recognition factor-like; Component of the cytosolic iron-sulfur protein assembly (CIA) complex, a multiprotein complex that mediates the incorporation of iron-sulfur cluster into extramitochondrial Fe/S proteins. Seems to negatively regulate the level of HIF1A expression, although this effect could be indirect (476 aa) | |||
ABCB7 | ATP-binding cassette, sub-family B (MDR/TAP), member 7; Could be involved in the transport of heme from the mitochondria to the cytosol. Plays a central role in the maturation of cytosolic iron-sulfur (Fe/S) cluster-containing proteins (753 aa) | |||
BRIP1 | BRCA1 interacting protein C-terminal helicase 1; DNA-dependent ATPase and 5’ to 3’ DNA helicase required for the maintenance of chromosomal stability. Acts late in the Fanconi anemia pathway, after FANCD2 ubiquitination. Involved in the repair of DNA double-strand breaks by homologous recombination in a manner that depends on its association with BRCA1 (1249 aa) | |||
NUBP2 | nucleotide binding protein 2; Component of the cytosolic iron-sulfur (Fe/S) protein assembly (CIA) machinery. Required for maturation of extramitochondrial Fe-S proteins. The NUBP1-NUBP2 heterotetramer forms a Fe-S scaffold complex, mediating the de novo assembly of an Fe-S cluster and its transfer to target apoproteins (By similarity) (271 aa) | |||
PDCD6 | programmed cell death 6; Calcium-binding protein required for T-cell receptor-, Fas-, and glucocorticoid-induced cell death. May mediate Ca(2+)- regulated signals along the death pathway (By similarity). Calcium-dependent adapter necessary for the association between PDCD6IP and TSG101. Interaction with DAPK1 can accelerate apoptotic cell death by increasing caspase-3 activity (191 aa) | |||
NUBP1 | nucleotide binding protein 1; Implicated in the regulation of centrosome duplication (By similarity). Component of the cytosolic iron-sulfur (Fe/S) protein assembly (CIA) machinery. Required for maturation of extramitochondrial Fe-S proteins. The NUBP1-NUBP2 heterotetramer forms a Fe-S scaffold complex, mediating the de novo assembly of an Fe-S cluster and its transfer to target apoproteins (320 aa) | |||
MRPL10 | mitochondrial ribosomal protein L10 (271 aa) | |||
ICT1 | immature colon carcinoma transcript 1; Essential peptidyl-tRNA hydrolase component of the mitochondrial large ribosomal subunit. Acts as a codon-independent translation release factor that has lost all stop codon specificity and directs the termination of translation in mitochondrion, possibly in case of abortive elongation. May be involved in the hydrolysis of peptidyl-tRNAs that have been prematurely terminated and thus in the recycling of stalled mitochondrial ribosomes (206 aa) | |||
UBC | ubiquitin C (685 aa) | |||
RTEL1 | regulator of telomere elongation helicase 1 (1219 aa) | |||
SLC25A28 | solute carrier family 25 (mitochondrial iron transporter), member 28; Mitochondrial iron transporter that mediates iron uptake. Probably required for heme synthesis of hemoproteins and Fe-S cluster assembly in non-erythroid cells. The iron delivered into the mitochondria, presumably as Fe(2+), is then probably delivered to ferrochelatase to catalyze Fe(2+) incorporation into protoprophyrin IX to make heme (By similarity) (364 aa) | |||
MMS19 | MMS19 nucleotide excision repair homolog (S. cerevisiae) (1030 aa) | |||
NDOR1 | NADPH dependent diflavin oxidoreductase 1; Oxidoreductase that catalyzes the NADP-dependent reduction of cytochrome c and one-electron acceptors, such as doxorubicin, potassium ferricyanide and menadione (in vitro) (606 aa) | |||
MTCH1 | mitochondrial carrier 1 (372 aa) | |||
NDUFB6 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (128 aa) | |||
FECH | ferrochelatase; Catalyzes the ferrous insertion into protoporphyrin IX (By similarity) (429 aa) | |||
ERCC2 | excision repair cross-complementing rodent repair deficiency, complementation group 2; ATP-dependent 5’-3’ DNA helicase, component of the core- TFIIH basal transcription factor. Involved in nucleotide excision repair (NER) of DNA by opening DNA around the damage, and in RNA transcription by RNA polymerase II by anchoring the CDK-activating kinase (CAK) complex, composed of CDK7, cyclin H and MAT1, to the core-TFIIH complex. Involved in the regulation of vitamin-D receptor activity. As part of the mitotic spindle-associated MMXD complex it plays a role in chromosome segregation. Might h [...] (760 aa) | |||
CIAPIN1 | cytokine induced apoptosis inhibitor 1; May be required for the maturation of extramitochondrial Fe/S proteins (By similarity). Has anti-apoptotic effects in the cell. Involved in negative control of cell death upon cytokine withdrawal. Promotes development of hematopoietic cells (By similarity) (312 aa) | |||
NSF | N-ethylmaleimide-sensitive factor; Required for vesicle-mediated transport. Catalyzes the fusion of transport vesicles within the Golgi cisternae. Is also required for transport from the endoplasmic reticulum to the Golgi stack. Seem to function as a fusion protein required for the delivery of cargo proteins to all compartments of the Golgi stack independent of vesicle origin. Interaction with AMPAR subunit GRIA2 leads to influence GRIA2 membrane cycling (By similarity) (744 aa) | |||
FAM96B | family with sequence similarity 96, member B; Component of the cytosolic iron-sulfur protein assembly (CIA) complex, a multiprotein complex that mediates the incorporation of iron-sulfur cluster into extramitochondrial Fe/S proteins. As part of the mitotic spindle-associated MMXD complex it plays a role in chromosome segregation, probably by facilitating iron-sulfur cluster assembly into ERCC2/XPD (163 aa) | |||
POLD1 | polymerase (DNA directed), delta 1, catalytic subunit; Possesses two enzymatic activities- DNA synthesis (polymerase) and an exonucleolytic activity that degrades single stranded DNA in the 3’- to 5’-direction. Required with its accessory proteins (proliferating cell nuclear antigen (PCNA) and replication factor C (RFC) or activator 1) for leading strand synthesis. Also involved in completing Okazaki fragments initiated by the DNA polymerase alpha/primase complex (1107 aa) | |||
CIAO1 | cytosolic iron-sulfur protein assembly 1; Key component of the cytosolic iron-sulfur protein assembly (CIA) complex, a multiprotein complex that mediates the incorporation of iron-sulfur cluster into extramitochondrial Fe/S proteins. Seems to specifically modulate the transactivation activity of WT1. As part of the mitotic spindle-associated MMXD complex it may play a role in chromosome segregation (339 aa) | |||
SLC25A37 | solute carrier family 25 (mitochondrial iron transporter), member 37; Mitochondrial iron transporter that specifically mediates iron uptake in developing erythroid cells, thereby playing an essential role in heme biosynthesis. The iron delivered into the mitochondria, presumably as Fe(2+), is then probably delivered to ferrochelatase to catalyze Fe(2+) incorporation into protoprophyrin IX to make heme (By similarity) (338 aa) |