NAD(P)H oxidoreductase RTN4IP1, mitochondrial (RTN4IP1)

NAD(P)H oxidoreductase RTN4IP1, mitochondrial (RTN4IP1) is a mitochondrial matrix enzyme that catalyzes electron transfer from NAD(P)H to quinone molecules, playing a central role in *coenzyme Q (CoQ) biosynthesis* and *mitochondrial complex I assembly*[1][2][6]. RTN4IP1 is essential for efficient mitochondrial respiration, especially in high-energy tissues such as muscle and neural tissue. Loss-of-function variants in RTN4IP1 cause *optic atrophy 10 (OPA10)*, a neurodegenerative disorder characterized by progressive vision loss, often accompanied by ataxia, seizures, developmental delay, and myopathy[3][5]. The protein also modulates neurite outgrowth via interaction with reticulon-4/NOGO, a known inhibitor of neural regeneration. RTN4IP1’s pivotal roles in mitochondrial physiology, neural development, and response to oxidative stress highlight its significance both in rare mitochondrial syndromes and potentially in tumorigenesis[3][5]. No specific drugs are currently known to target RTN4IP1, but coenzyme Q10 supplementation can mitigate some symptoms associated with its deficiency[1].

Other names

Optic atrophy 10 proteinOPA10NOGO-interacting mitochondrial proteinNIMPReticulon-4-interacting protein 1Reticulon 4 interacting protein 1

Not applicable as no drugs directly target RTN4IP1; however, coenzyme Q10 (CoQ10) supplementation may compensate for defects in CoQ biosynthesis secondary to RTN4IP1 dysfunction[1]