NDUFS7
适用: 小鼠
WB, IF (cc), IF (p)
宿主: 兔
Polyclonal
Alexa Fluor 750
应用备注
Peptide ELISA: Limit Dilution: 1/16000. Western blot: 1-3 μg/mL. This antibody detects a band of approx. 20 kDa in Human Heart andHuman Skeletal Muscle lysates. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
限制
仅限研究用
浓度
0.5 mg/mL
缓冲液
Tris saline, pH 7.3 containing 0.02 % Sodium Azide as preservative and 0.5 % BSA as stabilizer.
储存液
Sodium azide
注意事项
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
注意事项
Avoid repeated freezing and thawing.
储存条件
4 °C/-20 °C
储存方法
Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
Defects in NDUFS7 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in NDUFS7 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.Synonyms: CI-20kD, Complex I-20kD, NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, NADH-ubiquinone oxidoreductase 20 kDa subunit, PSST subunit, mitochondrial