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MIRAGE

SPG7 matrix AAA peptidase subunit, paraplegin

Summary

Gene Symbol

SPG7

Organism

Homo sapiens (human)

External Links

NCBI Gene

6687

PubChem

6687

Alliance of Genome Resources

HGNC:11237

Annotation

Keyword

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

Proteins

Displaying **all 2** entries
UniProt	Protein Name
A0A2R8Y632
Q9UQ90	Cell matrix adhesion regulator Paraplegin Spastic paraplegia 7 protein

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **6 - 7** of 7 in total

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GO Term	Evidence Code	PMID
mitochondrial outer membrane permeabilization involved in programmed cell death	IMP	26387735
mitochondrial protein processing	IBA

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

organonitrogen compound metabolic process [inference]

protein metabolic process [inference]

protein processing [inference]

mitochondrial protein processing

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing

primary metabolic process [inference]

protein metabolic process [inference]

protein processing [inference]

mitochondrial protein processing

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing

organic substance metabolic process [inference]

macromolecule metabolic process [inference]

protein metabolic process [inference]

protein processing [inference]

mitochondrial protein processing

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing

organonitrogen compound metabolic process [inference]

protein metabolic process [inference]

protein processing [inference]

mitochondrial protein processing

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing

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

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

cellular component organization or biogenesis [inference]

cellular component organization [inference]

organelle organization [inference]

mitochondrion organization [inference]

mitochondrial membrane organization [inference]

regulation of mitochondrial membrane permeability

positive regulation of mitochondrial membrane permeability [inference]

mitochondrial outer membrane permeabilization involved in programmed cell death

membrane organization [inference]

mitochondrial membrane organization [inference]

regulation of mitochondrial membrane permeability

positive regulation of mitochondrial membrane permeability [inference]

mitochondrial outer membrane permeabilization involved in programmed cell death

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

Displaying **all 5** entries
GO Term	Evidence Code	PMID
m-AAA complex	IBA IDA	28396416
mitochondrial permeability transition pore complex	IDA	26387735
mitochondrial inner membrane	IDA ISS TAS	9635427
mitochondrion	HTP	34800366
axon cytoplasm	IEA

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 entity [inference]

cytoplasm [inference]

cytoplasmic region [inference]

plasma membrane bounded cell projection 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 **6 - 8** of 8 in total

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GO Term	Evidence Code	PMID
protein binding	IPI	14623864 16189514 21516116 25416956 26387735 28514442 32296183 33961781
ATP hydrolysis activity	IEA
metalloendopeptidase activity	IBA IDA	28396416 31097542

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

unfolded protein binding

small molecule binding [inference]

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

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

organic cyclic compound binding [inference]

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

carbohydrate derivative binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ATP-dependent activity [inference]

ATP-dependent peptidase activity

Gene Ontology information from NCBI Gene and UniProt.

Disease

Disease Ontology

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

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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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Last updated: February 17, 2025