Function
Locations
Thioredoxin-fold peripheral-arm accessory subunit (non-catalytic).
Beyond its 14 conserved "core" subunits, mitochondrial respiratory Complex I (NADH:ubiquinone oxidoreductase) carries ~30 nuclear-encoded accessory (supernumerary) subunits that form a shell around the core L-shaped enzyme. These subunits are stable constituents of the mature holoenzyme (unlike the transient assembly factors) but are non-catalytic — they carry no redox cofactor and do not themselves perform the NADH:ubiquinone reaction. Their roles are structural: they stabilise and shield the core, seal the membrane arm, and are required for assembly and stability of a fully active complex. This module groups the human accessory subunits by which arm they decorate. The peripheral (matrix) arm accessory subunits — NDUFA2 (thioredoxin-fold), NDUFA5, NDUFA6, NDUFA12 (near the N/Q junction) and NDUFA13/GRIM-19 — buttress the flavoprotein and Q modules; NDUFA13 additionally has a well-documented moonlighting role as a negative regulator of STAT3 signalling and in apoptosis, curated separately from its structural role. The membrane arm accessory subunits — NDUFA1 (MWFE), NDUFA8 (twin-CX9C), NDUFA10, NDUFA11 (Tim17-like fold), NDUFB3, NDUFB7, NDUFB8, NDUFB9, NDUFB10 and NDUFB11 (several imported and disulfide-folded by the MIA40/CHCHD4 relay), and NDUFC2 — clamp the hydrophobic ND subunits. Loss of individual accessory subunits destabilises the holoenzyme and causes isolated mitochondrial complex I deficiency (Leigh syndrome, cardiomyopathy, leukoencephalopathy; NDUFB11 is X-linked, causing histiocytoid cardiomyopathy / MLS-like disease).
All recommended fields populated.
✗ none found
No MODULE:complex_i_accessory_subunits deep-research report alongside the module YAML.
✓ every leaf node grounds to a representative protein.
✓ every declared conforms_to bundle matches its template motif.
16 gene-function check(s) reflected in core functions · 0 conflict(s) · 16 evidence or coverage gap(s) (32 comparison(s))
Retained noncore functions are reported separately; they do not require promotion to core functions. Support from representative genes does not establish conservation across the whole family. Missing reviews or identifiers leave a claim unverified.
| Annoton | Participant | Function | Assessment |
|---|---|---|---|
| ndufa2_activity | NDUFA2 (human) UniProtKB:O43678 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa5_activity | NDUFA5 (human) UniProtKB:Q16718 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa6_activity | NDUFA6 (human) UniProtKB:P56556 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa12_activity | NDUFA12 (human) UniProtKB:Q9UI09 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa13_activity | NDUFA13 (human) UniProtKB:Q9P0J0 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa1_activity | NDUFA1 (human) UniProtKB:O15239 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa8_activity | NDUFA8 (human) UniProtKB:P51970 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa10_activity | NDUFA10 (human) UniProtKB:O95299 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa11_activity | NDUFA11 (human) UniProtKB:Q86Y39 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufb3_activity | NDUFB3 (human) UniProtKB:O43676 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufb7_activity | NDUFB7 (human) UniProtKB:P17568 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufb8_activity | NDUFB8 (human) UniProtKB:O95169 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufb9_activity | NDUFB9 (human) UniProtKB:Q9Y6M9 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufb10_activity | NDUFB10 (human) UniProtKB:O96000 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufb11_activity | NDUFB11 (human) UniProtKB:Q9NX14 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufc2_activity | NDUFC2 (human) UniProtKB:O95298 Family Representative |
structural molecule activity GO:0005198 |
Core Supported
The GO molecular function is reflected in core_functions (exact term or a reviewed subclass). |
| ndufa2_activity | NDUFA2 / B8 thioredoxin-fold accessory family PANTHER:PTHR12878 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR12878. |
| ndufa5_activity | NDUFA5 / B13 accessory family PANTHER:PTHR12653 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR12653. |
| ndufa6_activity | NDUFA6 / B14 (LYRM6) accessory family PANTHER:PTHR12964 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR12964. |
| ndufa12_activity | NDUFA12 / B17.2 accessory family PANTHER:PTHR12910 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR12910. |
| ndufa13_activity | NDUFA13 / GRIM-19 accessory family PANTHER:PTHR12966 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR12966. |
| ndufa1_activity | NDUFA1 / MWFE accessory family PANTHER:PTHR17098 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR17098. |
| ndufa8_activity | NDUFA8 / PGIV twin-CX9C accessory family PANTHER:PTHR13344 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR13344. |
| ndufa10_activity | NDUFA10 / deoxyribonucleoside-kinase-fold accessory family PANTHER:PTHR10513 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR10513. |
| ndufa11_activity | NDUFA11 / B14.7 Tim17-fold accessory family PANTHER:PTHR21382 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR21382. |
| ndufb3_activity | NDUFB3 / B12 accessory family PANTHER:PTHR15082 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR15082. |
| ndufb7_activity | NDUFB7 / B18 twin-CX9C accessory family PANTHER:PTHR20900 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR20900. |
| ndufb8_activity | NDUFB8 / ASHI accessory family PANTHER:PTHR12840 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR12840. |
| ndufb9_activity | NDUFB9 / B22 (LYRM3) accessory family PANTHER:PTHR12868 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR12868. |
| ndufb10_activity | NDUFB10 / PDSW twin-CX9C accessory family PANTHER:PTHR13094 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR13094. |
| ndufb11_activity | NDUFB11 / ESSS accessory family PANTHER:PTHR13327 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR13327. |
| ndufc2_activity | NDUFC2 / B14.5b accessory family PANTHER:PTHR13099 Family |
structural molecule activity GO:0005198 |
Family Review Missing
No supported structured family review is available for PANTHER:PTHR13099. |
16 complete review(s) · 0 with deep research · 0 missing review · 16 reviewed but lacking deep research
| Gene | Review | Complete | Deep research |
|---|---|---|---|
| NDUFA1 O15239 | ✓ | ✓ | ✗ |
| NDUFA10 O95299 | ✓ | ✓ | ✗ |
| NDUFA11 Q86Y39 | ✓ | ✓ | ✗ |
| NDUFA12 Q9UI09 | ✓ | ✓ | ✗ |
| NDUFA13 Q9P0J0 | ✓ | ✓ | ✗ |
| NDUFA2 O43678 | ✓ | ✓ | ✗ |
| NDUFA5 Q16718 | ✓ | ✓ | ✗ |
| NDUFA6 P56556 | ✓ | ✓ | ✗ |
| NDUFA8 P51970 | ✓ | ✓ | ✗ |
| NDUFB10 O96000 | ✓ | ✓ | ✗ |
| NDUFB11 Q9NX14 | ✓ | ✓ | ✗ |
| NDUFB3 O43676 | ✓ | ✓ | ✗ |
| NDUFB7 P17568 | ✓ | ✓ | ✗ |
| NDUFB8 O95169 | ✓ | ✓ | ✗ |
| NDUFB9 Q9Y6M9 | ✓ | ✓ | ✗ |
| NDUFC2 O95298 | ✓ | ✓ | ✗ |
Accessory/supernumerary (non-core) subunits of mitochondrial respiratory Complex I (NADH:ubiquinone oxidoreductase), grouped by arm and grounded to the completed human gene reviews. Peripheral (matrix) arm: NDUFA2 (O43678), NDUFA5 (Q16718), NDUFA6 (P56556), NDUFA12 (Q9UI09), NDUFA13 (Q9P0J0, GRIM-19). Membrane arm: NDUFA1 (O15239), NDUFA8 (P51970), NDUFA10 (O95299), NDUFA11 (Q86Y39), NDUFB3 (O43676), NDUFB7 (P17568), NDUFB8 (O95169), NDUFB9 (Q9Y6M9), NDUFB10 (O96000), NDUFB11 (Q9NX14), NDUFC2 (O95298). Every member is a non-catalytic structural constituent (GO:0005198 structural molecule activity) that contributes to, but does not itself enable, the complex-level NADH dehydrogenase (ubiquinone) activity (GO:0008137); all are part_of respiratory chain complex I (GO:0045271) at the mitochondrial inner membrane (GO:0005743). Notable subunits: NDUFA10 retains a deoxyribonucleoside-kinase fold but is catalytically inactive (pseudokinase — no kinase MF assigned); NDUFA13/GRIM-19 has a genuine STAT3-inhibition/apoptosis moonlighting role that is curated as a separate non-core function in its gene review; several (NDUFA8/NDUFB7/NDUFB10) are twin-CX9C proteins imported by the MIA40/CHCHD4 disulfide relay. This module complements the merged mitochondrial_complex_i_core (catalytic N+Q modules) and complex_i_assembly_factors modules; together they cover the nuclear-encoded Complex I proteome (the mtDNA-encoded ND1-6/ND4L membrane subunits are curated separately). GO term ids/labels were verified against the local go.db. Disorders: isolated mitochondrial complex I deficiency — Leigh syndrome, cardiomyopathy, leukoencephalopathy; NDUFB11 is X-linked (histiocytoid cardiomyopathy / MLS-like).
Thioredoxin-fold peripheral-arm accessory subunit (non-catalytic).
Peripheral-arm accessory subunit (non-catalytic).
Peripheral-arm accessory subunit near the Q site (non-catalytic).
N/Q-module junction accessory subunit (non-catalytic; not the NDUFAF2 assembly factor).
Peripheral-arm accessory subunit (GRIM-19; STAT3/apoptosis moonlighting is a separate non-core role).
MWFE membrane-arm accessory subunit (non-catalytic).
Twin-CX9C membrane-arm accessory subunit (non-catalytic).
dNK-fold (catalytically inactive) membrane-arm accessory subunit.
Tim17-fold membrane-arm accessory subunit (non-catalytic).
Membrane-arm accessory subunit (non-catalytic).
Twin-CX9C membrane-arm accessory subunit (non-catalytic).
Membrane-arm accessory subunit (non-catalytic).
LYR-family membrane-arm accessory subunit (non-catalytic).
Twin-CX9C membrane-arm accessory subunit (non-catalytic).
X-linked membrane-arm accessory subunit (non-catalytic).
Membrane-arm accessory subunit (non-catalytic).