The TTC19 Polyclonal Antibody (PAC053106) is a valuable tool for researchers studying the TTC19 protein, which is involved in mitochondrial function and energy production. This antibody, generated in rabbits, exhibits high specificity and sensitivity for detecting TTC19 in human samples, making it suitable for various research applications such as Western blotting.TTC19 is a crucial player in mitochondrial biology, playing a role in the assembly of complex III in the mitochondrial respiratory chain. Dysregulation of TTC19 has been linked to mitochondrial dysfunction and energy metabolism disorders, making it a potential target for therapeutic interventions in conditions such as mitochondrial diseases and neurodegenerative disorders.
By using the TTC19 Polyclonal Antibody, researchers can gain valuable insights into the function and regulation of the TTC19 protein in different cell types and tissues, paving the way for advancements in the understanding and treatment of mitochondrial-related disorders.
Immunohistochemistry of paraffin-embedded human cervical cancer using PACO53106 at dilution of 1:100.
Immunofluorescent analysis of MCF-7 cells using PACO53106 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
Background:
Mitochondrial protein required for formation of the mitochondrial complex III. May also be required for the abcission step in cytokinesis, possibly regulating the ESCRT-III complex via its interaction with CHMP4B. However, the involvement in cytokinesis requires additional experimental evidence.
Synonyms:
Tetratricopeptide repeat protein 19, mitochondrial (TPR repeat protein 19), TTC19
UniProt Protein Function:
TTC19: Mitochondrial protein required for formation of the mitochondrial complex III (PubMed:21278747). May also be required for the abcission step in cytokinesis, possibly regulating the ESCRT-III complex via its interaction with CHMP4B (PubMed:20208530). However, the involvement in cytokinesis requires additional experimental evidence. Defects in TTC19 are a cause of mitochondrial complex III deficiency (MT-C3D). A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. Belongs to the TTC19 family.Protein type: MitochondrialChromosomal Location of Human Ortholog: 17p12Cellular Component: centrosome; mitochondrion; mitochondrial inner membrane; midbodyMolecular Function: protein bindingBiological Process: cytokinesisDisease: Mitochondrial Complex Iii Deficiency, Nuclear Type 2
UniProt Protein Details:
NCBI Summary:
This gene encodes a protein with a tetratricopeptide repeat (TPR) domain containing several TPRs of about 34 aa each. These repeats are found in a variety of organisms including bacteria, fungi and plants, and are involved in a variety of functions including protein-protein interactions. This protein is embedded in the inner mitochondrial membrane and is involved in the formation of the mitochondrial respiratory chain III. It has also been suggested that this protein plays a role in cytokinesis. Mutations in this gene cause mitochondrial complex III deficiency. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Sep 2012]