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Mitochondrial inner membrane m-AAA protease component paraplegin

Summary

UniProt ID: Q9UQ90
Gene Symbol: SPG7 CAR CMAR PGN
Gene ID: 6687
Organism: Homo sapiens (human)

External Links

GlyGen: Q9UQ90
PubChem: Q9UQ90
Re-Glyco: Q9UQ90

Annotation

Keyword: 3D-structure ATP-binding Alternative splicing Disease variant Hereditary spastic paraplegia Metal-binding Metalloprotease Mitochondrion inner membrane Nitration Osteogenesis imperfecta Proteomics identification Reference proteome Transit peptide Transmembrane helix Zinc

Gene Ontology (GO)

Displaying entries **1 - 5** of 11 in total

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GO Term	Evidence Code	Reference
proteolysis	TAS	PMID
proteolysis	IEA	GO_REF
nervous system development	TAS	PMID
anterograde axonal transport
mitochondrial protein processing	IDA	PMID

GO Hierarchy

biological process

cellular process [inference]

microtubule-based process [inference]

microtubule-based movement [inference]

transport along microtubule [inference]

axo-dendritic transport [inference]

axonal transport [inference]

anterograde axonal transport

metabolic process [inference]

macromolecule metabolic process [inference]

protein metabolic process [inference]

proteolysis

protein processing [inference]

mitochondrial protein processing

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing

primary metabolic process [inference]

protein metabolic process [inference]

proteolysis

protein processing [inference]

mitochondrial protein processing

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing

cellular localization [inference]

intracellular transport [inference]

cytoskeleton-dependent intracellular transport [inference]

transport along microtubule [inference]

axo-dendritic transport [inference]

axonal transport [inference]

anterograde axonal transport

developmental process [inference]

anatomical structure development [inference]

system development [inference]

nervous system development

localization [inference]

establishment of localization [inference]

transport [inference]

intracellular transport [inference]

cytoskeleton-dependent intracellular transport [inference]

transport along microtubule [inference]

axo-dendritic transport [inference]

axonal transport [inference]

anterograde axonal transport

microtubule-based transport [inference]

transport along microtubule [inference]

axo-dendritic transport [inference]

axonal transport [inference]

anterograde axonal transport

establishment of localization in cell [inference]

intracellular transport [inference]

cytoskeleton-dependent intracellular transport [inference]

transport along microtubule [inference]

axo-dendritic transport [inference]

axonal transport [inference]

anterograde axonal transport

cellular localization [inference]

intracellular transport [inference]

cytoskeleton-dependent intracellular transport [inference]

transport along microtubule [inference]

axo-dendritic transport [inference]

axonal transport [inference]

anterograde axonal transport

biological regulation [inference]

regulation of biological process [inference]

regulation of localization [inference]

regulation of transport [inference]

regulation of transmembrane transport [inference]

regulation of monoatomic ion transmembrane transport [inference]

regulation of monoatomic cation transmembrane transport [inference]

regulation of calcium ion transmembrane transport [inference]

regulation of calcium import into the mitochondrion

regulation of monoatomic ion transport [inference]

regulation of metal ion transport [inference]

regulation of calcium ion transport [inference]

regulation of calcium ion transmembrane transport [inference]

regulation of calcium import into the mitochondrion

regulation of monoatomic ion transmembrane transport [inference]

regulation of monoatomic cation transmembrane transport [inference]

regulation of calcium ion transmembrane transport [inference]

regulation of calcium import into the mitochondrion

regulation of cellular process [inference]

regulation of transmembrane transport [inference]

regulation of monoatomic ion transmembrane transport [inference]

regulation of monoatomic cation transmembrane transport [inference]

regulation of calcium ion transmembrane transport [inference]

regulation of calcium import into the mitochondrion

regulation of biological quality [inference]

regulation of membrane permeability [inference]

regulation of mitochondrial membrane permeability

positive regulation of mitochondrial membrane permeability [inference]

mitochondrial outer membrane permeabilization involved in programmed cell death

positive regulation of membrane permeability [inference]

positive regulation of mitochondrial membrane permeability [inference]

mitochondrial outer membrane permeabilization involved in programmed cell death

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GO Term	Evidence Code	Reference
mitochondrion	TAS	PMID
mitochondrion	IEA	GO_REF
mitochondrion	HTP	PMID
mitochondrial inner membrane	ISS	GO_REF
mitochondrial inner membrane	TAS	Reactome Reactome Reactome Reactome Reactome

GO Hierarchy

cellular component

protein-containing complex [inference]

membrane protein complex [inference]

pore complex [inference]

mitochondrial permeability transition pore complex

inner mitochondrial membrane protein complex [inference]

m-AAA complex

mitochondrial protein-containing complex [inference]

mitochondrial permeability transition pore complex

inner mitochondrial membrane protein complex [inference]

m-AAA complex

catalytic complex [inference]

peptidase complex [inference]

m-AAA complex

cellular anatomical structure [inference]

cytoplasm [inference]

neuron projection cytoplasm [inference]

axon cytoplasm

membrane [inference]

organelle membrane [inference]

organelle inner membrane [inference]

mitochondrial inner membrane

mitochondrial membrane [inference]

mitochondrial inner membrane

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

Displaying entries **1 - 5** of 10 in total

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GO Term	Evidence Code	Reference
ATP-dependent peptidase activity	IEA	GO_REF
metalloendopeptidase activity	IDA	PMID PMID PMID
metalloendopeptidase activity	IBA	GO_REF
metalloendopeptidase activity	IEA	GO_REF
ATP binding	IEA	GO_REF

GO Hierarchy

molecular function

catalytic activity [inference]

hydrolase activity [inference]

peptidase activity

endopeptidase activity [inference]

metalloendopeptidase activity

ATP-dependent peptidase activity

metallopeptidase activity [inference]

metalloendopeptidase activity

hydrolase activity, acting on acid anhydrides [inference]

hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides [inference]

pyrophosphatase activity [inference]

ribonucleoside triphosphate phosphatase activity [inference]

ATP hydrolysis activity

catalytic activity, acting on a protein [inference]

peptidase activity

endopeptidase activity [inference]

metalloendopeptidase activity

ATP-dependent peptidase activity

metallopeptidase activity [inference]

metalloendopeptidase activity

binding [inference]

protein binding [inference]

unfolded protein binding

small molecule binding [inference]

ion binding [inference]

anion binding [inference]

purine ribonucleoside triphosphate binding [inference]

ATP binding

cation binding [inference]

metal ion binding [inference]

transition metal ion binding [inference]

zinc ion binding

heterocyclic compound binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleoside triphosphate binding [inference]

ATP binding

carbohydrate derivative binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

nucleoside phosphate binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleoside triphosphate binding [inference]

ATP binding

ATP-dependent activity [inference]

ATP-dependent peptidase activity

Gene Ontology information from UniProt.

Annotation information from UniProt.

Sequence

MAVLLLLLRALRRGPGPGPRPLWGPGPAWSPGFPARPGRGRPYMASRPPGDLAEAGGRALQSLQLRLLTPTFEGINGLLLKQHLVQNPVRLWQLLGGTFYFNTSRLKQKNKEKDKSKGKAPEEDEEERRRRERDDQMYRERLRTLLVIAVVMSLLNALSTSGGSISWNDFVHEMLAKGEVQRVQVVPESDVVEVYLHPGAVVFGRPRLALMYRMQVANIDKFEEKLRAAEDELNIEAKDRIPVSYKRTGFFGNALYSVGMTAVGLAILWYVFRLAGMTGREGGFSAFNQLKMARFTIVDGKMGKGVSFKDVAGMHEAKLEVREFVDYLKSPERFLQLGAKVPKGALLLGPPGCGKTLLAKAVATEAQVPFLAMAGPEFVEVIGGLGAARVRSLFKEARARAPCIVYIDEIDAVGKKRSTTMSGFSNTEEEQTLNQLLVEMDGMGTTDHVIVLASTNRADILDGALMRPGRLDRHVFIDLPTLQERREIFEQHLKSLKLTQSSTFYSQRLAELTPGFSGADIANICNEAALHAAREGHTSVHTLNFEYAVERVLAGTAKKSKILSKEEQKVVAFHESGHALVGWMLEHTEAVMKVSITPRTNAALGFAQMLPRDQHLFTKEQLFERMCMALGGRASEALSFNEVTSGAQDDLRKVTRIAYSMVKQFGMAPGIGPISFPEAQEGLMGIGRRPFSQGLQQMMDHEARLLVAKAYRHTEKVLQDNLDKLQALANALLEKEVINYEDIEALIGPPPHGPKKMIAPQRWIDAQREKQDLGEEETEETQQPPLGGEEPTWPK

N : N-glycosylation Site
___ : Potential Sequon

Glycosylation Sites

Displaying 1 entry
Position	Description	PubMed ID	GlyTouCan ID	Source
792				GlyGen

Feature

: Glycosylation Site from GlyGen
: Glycosylation Site from UniProt

Pathway

Displaying **all 2** entries
Pathway Name	Organism
Mitochondrial protein degradation	Homo sapiens
Processing of SMDT1	Homo sapiens

Disease

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0110816	hereditary spastic paraplegia 7	Alliance of Genome Resources

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

LM-GlycoRepo

All Resources

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Acknowledgements