HTRA2

This gene encodes a serine protease. The protein has been localized in the endoplasmic reticulum and interacts with an alternatively spliced form of mitogen-activated protein kinase 14. The protein has also been localized to the mitochondria with release to the cytosol following apoptotic stimulus. The protein is thought to induce apoptosis by binding the apoptosis inhibitory protein baculoviral IAP repeat-containing 4. Nuclear localization of this protein has also been observed. Alternate splicing of this gene results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Mar 2016]

HtrA Serine Peptidase 2

Serine protease that shows proteolytic activity against a non-specific substrate beta-casein. Promotes or induces cell death either by direct binding to and inhibition of BIRC proteins (also called inhibitor of apoptosis proteins, IAPs), leading to an increase in caspase activity, or by a BIRC inhibition-independent, caspase-independent and serine protease activity-dependent mechanism. Cleaves THAP5 and promotes its degradation during apoptosis. Isoform 2 seems to be proteolytically inactive.

Adult walking behavior Source: Ensembl
Aging Source: Ensembl
Cellular protein catabolic process Source: ParkinsonsUK-UCL
Cellular response to growth factor stimulus Source: UniProtKB
Cellular response to heat Source: UniProtKB
Cellular response to interferon-beta Source: ParkinsonsUK-UCL
Cellular response to oxidative stress Source: ParkinsonsUK-UCL
Cellular response to retinoic acid Source: ParkinsonsUK-UCL
Ceramide metabolic process Source: Ensembl
Execution phase of apoptosis Source: UniProtKB
Forebrain development Source: Ensembl
Intrinsic apoptotic signaling pathway in response to DNA damage Source: ParkinsonsUK-UCL
Mitochondrion organization Source: Ensembl
Negative regulation of cell cycle Source: UniProtKB
Negative regulation of mitophagy in response to mitochondrial depolarization Source: Ensembl
Negative regulation of neuron death Source: ParkinsonsUK-UCL
Negative regulation of oxidative stress-induced intrinsic apoptotic signaling pathway Source: ParkinsonsUK-UCL
Neuron development Source: Ensembl
Pentacyclic triterpenoid metabolic process Source: Ensembl
Positive regulation of apoptotic process Source: UniProtKB
Positive regulation of cysteine-type endopeptidase activity involved in apoptotic process Source: UniProtKB
Positive regulation of cysteine-type endopeptidase activity involved in apoptotic signaling pathway Source: ParkinsonsUK-UCL
Positive regulation of extrinsic apoptotic signaling pathway in absence of ligand Source: UniProtKB
Positive regulation of mitochondrion organization Source: ParkinsonsUK-UCL
Positive regulation of protein targeting to mitochondrion Source: ParkinsonsUK-UCL
Programmed cell death Source: UniProtKB
Protein autoprocessing Source: ParkinsonsUK-UCL
Proteolysis Source: ParkinsonsUK-UCL
Regulation of autophagy of mitochondrion Source: ParkinsonsUK-UCL
Regulation of multicellular organism growth Source: Ensembl
Response to herbicide Source: Ensembl

Mitochondrion intermembrane space; Mitochondrion membrane. Predominantly present in the intermembrane space. Released into the cytosol following apoptotic stimuli, such as UV treatment, and stimulation of mitochondria with caspase-8 truncated BID/tBID.
Isoform 1: Endoplasmic reticulum

3-methylglutaconic aciduria 8 (MGCA8):
An autosomal recessive inborn error of metabolism resulting in early death. Clinical features include extreme hypertonia observed at birth, alternating with hypotonia, subsequent appearance of extrapyramidal symptoms, lack of psychomotor development, microcephaly, and intractable seizures. Patients show lactic acidemia, 3-methylglutaconic aciduria, intermittent neutropenia, and progressive brain atrophy.
Parkinson disease 13 (PARK13):
A complex neurodegenerative disorder characterized by bradykinesia, resting tremor, muscular rigidity and postural instability, as well as by a clinically significant response to treatment with levodopa. The pathology involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain.

Helical: 105-125

Autoproteolytically activated.