ELK Biotechnology MT-ATP8 Polyclona Antibody

MT-ATP8 Polyclonal Antibody

제품 개요

ELK Biotechnology에서 제공하는 MT-ATP8 단백질에 대한 rabbit polyclonal antibody입니다. 미토콘드리아 막 ATP synthase 복합체 연구에 적합하며, IHC 및 ELISA 실험에 활용 가능합니다.

제품 정보

Background

Defects in MT-ATP6 are associated with several mitochondrial disorders including:

• Infantile bilateral striatal necrosis (MIM:500003)
• Leber hereditary optic neuropathy (LHON, MIM:535000)
• Leigh syndrome (LS, MIM:256000)
• Neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP, MIM:551500)

Defects in MT-CO3 are linked to:

• Cytochrome c oxidase deficiency (COX deficiency, MIM:220110)
• Leber hereditary optic neuropathy (LHON, MIM:535000)
• Recurrent myoglobinuria (MIM:550500)
• Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS)

Function

Mitochondrial membrane ATP synthase (F₁F₀ ATP synthase or Complex V) produces ATP from ADP using a proton gradient generated by the respiratory chain.

• F-type ATPases consist of two domains: F₁ (catalytic core) and F₀ (membrane proton channel).
• ATP synthesis in F₁ is coupled via a rotary mechanism to proton translocation through F₀.
• MT-ATP8 is a minor subunit located with subunit a in the membrane, contributing to proton translocation.

Similarity

• Belongs to the ATPase A chain family
• Belongs to the ATPase protein 8 family
• Belongs to the cytochrome c oxidase subunit 3 family

Subunit Structure

F-type ATPases have two components:

• CF(1): catalytic core (subunits α₃, β₃, γ₁, δ₁, ε₁)
• CF(0): membrane proton channel (subunits a, b, c)