MITOCHONDRIAL PROTEIN IMPORT PATHWAY (PW:0001962)

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Description

The double membrane-bound mitochondria organelles play essential roles in cellular homeostasis. In addition to the major function of ATP production in the oxidative phosphorylation (OXPHOS) pathway, mitochondria are the site of other important metabolic pathway such as fatty acid oxidation, citric acid cycle and urea cycle, heme and iron-sulfur cluster biosynthesis. They also provide for calcium storage and signaling, and have a central role in apoptosis. The mitochondrial distribution/mass var

Pathway Diagram:

Elsevier Inc. cardiolipin ADP ATP respiratory complex IV ATP ---> ADP motor PAM beta-barrel precursor ---> inserted beta-barrel TIM23 complex ---- TOM complex carrier precursor ---> inserted carrier beta-barrel precursor carrier precursor presequence precursor cysteine-rich precursor alpha-helical precursor cysteine-rich precursor ---> oxidized, folded precursor MINOS system ---- TOM complex TOM complex MINOS system ---- SAM complex SAM complex small Tim proteins SAM complex ---- TOM complex IM peptidases inserted beta-barrel MIA system TIM23 complex Gfer ---> MIA system Gfer ---- MIA system MIA system ---> oxidized, folded precursor presequence precursor ---> inserted precursor IM peptidases ---> inserted precursor presequence precursor ---> released precursor oxidized, folded precursor inserted precursor Oxa1l mt-precursor ---> inserted precursor mt-precursor inserted alpha-helical alpha-helical precursor ---> inserted alpha-helical inserted carrier Gfer Gfer ---> respiratory complex IV TIM22 complex intermembrane space assembly pathway of mitochondrial protein import alpha-helical insertion pathway of mitochondrial protein import presequence pathway of mitochondrial protein import carrier pathway of mitochondrial protein import beta-barrel pathway of mitochondrial protein import electron transport chain pathway matrix peptidases ---> released precursor released precursor matrix peptidases motor PAM ---- TIM23 complex ATP ---- motor PAM cardiolipin ---- motor PAM cardiolipin ---- MINOS system MINOS system MIM system
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Genes in Pathway:

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mitochondrial protein import pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Apoo apolipoprotein O JBrowse link X 63,521,033 63,625,134 RGD:10412671
G Apool apolipoprotein O-like JBrowse link X 83,678,269 83,750,550 RGD:10412671
G Chchd3 coiled-coil-helix-coiled-coil-helix domain containing 3 JBrowse link 4 60,080,536 60,358,592 RGD:10412671
G Chchd6 coiled-coil-helix-coiled-coil-helix domain containing 6 JBrowse link 4 121,340,952 121,565,222 RGD:10412671
G Immt inner membrane mitochondrial protein JBrowse link 4 99,753,446 99,789,921 RGD:10412671
G Minos1 mitochondrial inner membrane organizing system 1 JBrowse link 5 157,546,855 157,573,183 RGD:10412671
G Oxa1l OXA1L, mitochondrial inner membrane protein JBrowse link 15 33,004,957 33,012,584 RGD:10412658
alpha-helical insertion pathway of mitochondrial protein import term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Tomm70 translocase of outer mitochondrial membrane 70 JBrowse link 11 45,477,053 45,511,409 RGD:10412658
beta-barrel pathway of mitochondrial protein import term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Mtx1 Metaxin 1 JBrowse link 2 188,522,605 188,528,747 RGD:10412662
G Mtx2 metaxin 2 JBrowse link 3 61,756,109 61,818,999 RGD:10412662
G Samm50 SAMM50 sorting and assembly machinery component JBrowse link 7 125,058,966 125,082,506 RGD:10412662
G Timm10 translocase of inner mitochondrial membrane 10 JBrowse link 3 72,226,563 72,230,087 RGD:10412662
G Timm13 translocase of inner mitochondrial membrane 13 JBrowse link 7 11,677,340 11,678,443 RGD:10412662
G Timm8a1 translocase of inner mitochondrial membrane 8 homolog A1 (yeast) JBrowse link X 105,351,714 105,355,722 RGD:10412662
G Timm8b translocase of inner mitochondrial membrane 8 homolog B JBrowse link 8 55,037,750 55,039,130 RGD:10412662
G Timm9 translocase of inner mitochondrial membrane 9 JBrowse link 6 93,549,484 93,562,314 RGD:10412662
G Tomm20 translocase of outer mitochondrial membrane 20 JBrowse link 19 59,863,421 59,873,411 RGD:10412662
G Tomm22 translocase of outer mitochondrial membrane 22 JBrowse link 7 120,901,934 120,903,684 RGD:10412662
G Tomm40 translocase of outer mitochondrial membrane 40 JBrowse link 1 80,617,749 80,630,253 RGD:10412662
G Tomm5 translocase of outer mitochondrial membrane 5 JBrowse link 5 60,655,708 60,658,568 RGD:10412662
G Tomm6 translocase of outer mitochondrial membrane 6 JBrowse link 9 15,313,346 15,314,872 RGD:10412662
G Tomm7 translocase of outer mitochondrial membrane 7 JBrowse link 4 7,835,949 7,842,790 RGD:10412662
G Tomm70 translocase of outer mitochondrial membrane 70 JBrowse link 11 45,477,053 45,511,409 RGD:10412662
carrier pathway of mitochondrial protein import. term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Timm10 translocase of inner mitochondrial membrane 10 JBrowse link 3 72,226,563 72,230,087 RGD:10412667
G Timm10b translocase of inner mitochondrial membrane 10B JBrowse link 1 170,471,265 170,474,111 RGD:10412667
G Timm22 translocase of inner mitochondrial membrane 22 JBrowse link 10 64,556,064 64,563,822 RGD:10412667
G Timm9 translocase of inner mitochondrial membrane 9 JBrowse link 6 93,549,484 93,562,314 RGD:10412667
G Tomm20 translocase of outer mitochondrial membrane 20 JBrowse link 19 59,863,421 59,873,411 RGD:10412661
G Tomm22 translocase of outer mitochondrial membrane 22 JBrowse link 7 120,901,934 120,903,684 RGD:10412661
G Tomm40 translocase of outer mitochondrial membrane 40 JBrowse link 1 80,617,749 80,630,253 RGD:10412661
G Tomm5 translocase of outer mitochondrial membrane 5 JBrowse link 5 60,655,708 60,658,568 RGD:10412661
G Tomm6 translocase of outer mitochondrial membrane 6 JBrowse link 9 15,313,346 15,314,872 RGD:10412661
G Tomm7 translocase of outer mitochondrial membrane 7 JBrowse link 4 7,835,949 7,842,790 RGD:10412661
G Tomm70 translocase of outer mitochondrial membrane 70 JBrowse link 11 45,477,053 45,511,409 RGD:10412661
intermembrane space assembly pathway of mitochondrial protein import term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Chchd4 coiled-coil-helix-coiled-coil-helix domain containing 4 JBrowse link 4 123,108,924 123,118,186 RGD:10412663
G Gfer growth factor, augmenter of liver regeneration JBrowse link 10 14,059,347 14,061,703 RGD:10412663
G Tomm40 translocase of outer mitochondrial membrane 40 JBrowse link 1 80,617,749 80,630,253 RGD:10412658
presequence pathway of mitochondrial protein import term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Dnajc19 DnaJ heat shock protein family (Hsp40) member C19 JBrowse link 2 120,503,093 120,531,782 RGD:10412659
G Grpel1 GrpE-like 1, mitochondrial JBrowse link 14 79,458,951 79,464,782 RGD:10412659
G Grpel2 GrpE-like 2, mitochondrial JBrowse link 18 57,037,995 57,050,234 RGD:10412659
G Hspa9 heat shock protein family A member 9 JBrowse link 18 27,731,072 27,749,235 RGD:10412659
G Immp1l inner mitochondrial membrane peptidase subunit 1 JBrowse link 3 95,955,126 96,024,316 RGD:10412669
G Immp2l inner mitochondrial membrane peptidase subunit 2 JBrowse link 6 60,958,351 61,859,457 RGD:10412669
G Mipep mitochondrial intermediate peptidase JBrowse link 15 41,084,180 41,192,621 RGD:10412669
G Pam16 presequence translocase associated motor 16 homolog JBrowse link 10 11,146,359 11,153,936 RGD:10412659
G Pmpca peptidase, mitochondrial processing alpha subunit JBrowse link 3 3,834,262 3,842,061 RGD:10412669
G Pmpcb peptidase, mitochondrial processing beta subunit JBrowse link 4 9,908,838 9,921,615 RGD:10412669
G Romo1 reactive oxygen species modulator 1 JBrowse link 3 151,688,213 151,689,813 RGD:10412658
RGD:10412659
G Timm17a translocase of inner mitochondrial membrane 17A JBrowse link 13 52,124,641 52,136,127 RGD:10412658
RGD:10412659
G Timm21 translocase of inner mitochondrial membrane 21 JBrowse link 18 81,803,165 81,807,627 RGD:10412658
RGD:10412659
G Timm23 translocase of inner mitochondrial membrane 23 JBrowse link 16 8,324,038 8,350,067 RGD:10412658
RGD:10412659
G Timm44 translocase of inner mitochondrial membrane 44 JBrowse link 12 2,517,006 2,533,707 RGD:10412659
G Timm50 translocase of inner mitochondrial membrane 50 JBrowse link 1 85,470,810 85,480,813 RGD:10412658
RGD:10412659
G Tomm20 translocase of outer mitochondrial membrane 20 JBrowse link 19 59,863,421 59,873,411 RGD:10412658
RGD:10412659
G Tomm22 translocase of outer mitochondrial membrane 22 JBrowse link 7 120,901,934 120,903,684 RGD:10412658
RGD:10412659
G Tomm40 translocase of outer mitochondrial membrane 40 JBrowse link 1 80,617,749 80,630,253 RGD:10412658
RGD:10412659
G Tomm5 translocase of outer mitochondrial membrane 5 JBrowse link 5 60,655,708 60,658,568 RGD:10412658
RGD:10412659
G Tomm6 translocase of outer mitochondrial membrane 6 JBrowse link 9 15,313,346 15,314,872 RGD:10412658
RGD:10412659
G Tomm7 translocase of outer mitochondrial membrane 7 JBrowse link 4 7,835,949 7,842,790 RGD:10412658
RGD:10412659
G Tomm70 translocase of outer mitochondrial membrane 70 JBrowse link 11 45,477,053 45,511,409 RGD:10412658
RGD:10412659
G Xpnpep3 X-prolyl aminopeptidase 3 JBrowse link 7 122,636,252 122,686,983 RGD:10412669

Pathway Gene Annotations

Disease Annotations Associated with Genes in the mitochondrial protein import pathway
Pathway Annotations Associated with Genes in the mitochondrial protein import pathway

References Associated with the mitochondrial protein import pathway:

Ontology Path Diagram:

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Import into Pathway Studio: