MT-ATP6 Blocking Peptide | ATP6 blocking peptide

MT-ATP6 Immunizing Peptide

Cat. Num. AAA428320

• Gene Names: MT-ATP6; ATPase6; MTATP6; ATP6
• Reactivity: Human
• Synonyms: MT-ATP6; MT-ATP6 Immunizing Peptide; ATP6; ATPase6; MTATP6; ATP6 blocking peptide

• Reactivity: Human
• Form/Format: 100ug of dried peptide
• Sequence: C-PTSKYLINNRLITTQ
• Sequence Length: 226

• Preparation and Storage: Shipped at ambient temperature, store at -20 degree C

NCBI and Uniprot Product Information

• NCBI GI #: 251831112
• NCBI GeneID: 4508
• NCBI Accession #: YP_003024031.1
• NCBI GenBank Nucleotide #: NC_012920.1
• Molecular Weight: 24,817 Da
• NCBI Official Full Name: ATP synthase F0 subunit 6 (mitochondrion)
• NCBI Official Synonym Full Names: mitochondrially encoded ATP synthase 6
• NCBI Official Symbol: MT-ATP6
• NCBI Official Synonym Symbols: ATPase6; MTATP6; ATP6
• NCBI Protein Information: ATP synthase F0 subunit 6
• UniProt Protein Name: ATP synthase subunit a
• UniProt Gene Name: MT-ATP6
• UniProt Synonym Gene Names: ATP6; ATPASE6; MTATP6
• UniProt Entry Name: ATP6_HUMAN

Uniprot Description

ATP6: Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Key component of the proton channel; it may play a direct role in the translocation of protons across the membrane. Defects in MT-ATP6 are the cause of neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP). Defects in MT-ATP6 are a cause of Leber hereditary optic neuropathy (LHON). LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Defects in MT-ATP6 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in MT-ATP6 are a cause of mitochondrial infantile bilateral striatal necrosis (MIBSN). Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome. Belongs to the ATPase A chain family.

Protein type: Membrane protein, integral; Mitochondrial; Transporter; Membrane protein, multi-pass; Transporter, ion channel

Chromosomal Location of Human Ortholog: -

Disease: Leber Optic Atrophy; Neuropathy, Ataxia, And Retinitis Pigmentosa