BCS1L Polyklonaler Antikörper

BCS1L Polyklonal Antikörper für IF, IHC, WB,ELISA

Wirt / Isotyp

Kaninchen / IgG

Getestete Reaktivität

human, Maus, Ratte

Anwendung

WB, IHC, IF,ELISA

Konjugation

Unkonjugiert

Publikationen

7

Kat-Nr. : 10175-2-AP

Synonyme

BCS1L, BJS, FLNMS, GRACILE, h BCS, h BCS1, Hs.6719, Mitochondrial chaperone BCS1, PTD

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Galerie der Validierungsdaten

• 

  WB analysis of A549 using 10175-2-AP

  A549 cells were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

• 

  WB analysis of mouse colon using 10175-2-AP

  mouse colon tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

• 

  WB analysis of mouse skeletal muscle using 10175-2-AP

  mouse skeletal muscle tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

• 

  WB analysis of rat liver using 10175-2-AP

  rat liver tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

• 

  WB analysis of mouse liver using 10175-2-AP

  mouse liver tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

• 

  WB analysis of mouse kidney using 10175-2-AP

  mouse kidney tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:500 incubated at room temperature for 1.5 hours.

• 

  WB analysis of mouse small intestine using 10175-2-AP

  mouse small intestine tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:600 incubated at room temperature for 1.5 hours.

• 

  WB analysis of human brain using 10175-2-AP

  human brain tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:500 incubated at room temperature for 1.5 hours.

• 

  WB analysis of HeLa using 10175-2-AP

  HeLa cells were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

• 

  WB analysis of HEK-293 using 10175-2-AP

  HEK-293 cells were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

• 

  IHC staining of human gliomas using 10175-2-AP

  Immunohistochemical analysis of paraffin-embedded human gliomas using 10175-2-AP (BCS1L antibody) at dilution of 1:50 (under 10x lens).

• 

  IHC staining of human kidney using 10175-2-AP

  Immunohistochemical analysis of paraffin-embedded human kidney using 10175-2-AP (BCS1L antibody) at dilution of 1:50 (under 10x lens).

• 

  IHC staining of human kidney using 10175-2-AP

  Immunohistochemical analysis of paraffin-embedded human kidney using 10175-2-AP (BCS1L antibody) at dilution of 1:50 (under 40x lens).

• 

  IF Staining of A549 using 10175-2-AP

  Immunofluorescent analysis of (10% Formaldehyde) fixed A549 cells using 10175-2-AP (BCS1L antibody) at dilution of 1:50 and Alexa Fluor 488-conjugated AffiniPure Goat Anti-Rabbit IgG(H+L).

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Geprüfte Anwendungen

• Erfolgreiche Detektion in WB: A549-Zellen, HEK-293-Zellen, HeLa-Zellen, humanes Hirngewebe, Maus-Dünndarmgewebe, Maus-Kolongewebe, Mauslebergewebe, Mausnierengewebe, Maus-Skelettmuskelgewebe, Rattenlebergewebe
• Erfolgreiche Detektion in IHC: humanes Gliomgewebe, humanes Nierengewebe; Hinweis: Antigendemaskierung mit TE-Puffer pH 9,0 empfohlen. (*) Wahlweise kann die Antigendemaskierung auch mit Citratpuffer pH 6,0 erfolgen.
• Erfolgreiche Detektion in IF: A549-Zellen

Empfohlene Verdünnung

• Western Blot (WB): WB : 1:500-1:1000
• Immunhistochemie (IHC): IHC : 1:20-1:200
• Immunfluoreszenz (IF): IF : 1:20-1:200

It is recommended that this reagent should be titrated in each testing system to obtain optimal results.

Sample-dependent, check data in validation data gallery

Veröffentlichte Anwendungen

• WB: See 7 publications below

Produktinformation

10175-2-AP bindet in WB, IHC, IF,ELISA BCS1L und zeigt Reaktivität mit human, Maus, Ratten

• Getestete Reaktivität: human, Maus, Ratte
• In Publikationen genannte Reaktivität: human
• Wirt / Isotyp: Kaninchen / IgG
• Klonalität: Polyklonal
• Typ: Antikörper
• Immunogen: BCS1L fusion protein Ag0231
• Vollständiger Name: BCS1-like (yeast)
• Berechnetes Molekulargewicht: 48 kDa
• Beobachtetes Molekulargewicht: 50-55 kDa
• GenBank-Zugangsnummer: BC007500
• Gene symbol: BCS1L
• Gene ID (NCBI): 617
• Konjugation: Unkonjugiert
• Form: Liquid
• Reinigungsmethode: Antigen-Affinitätsreinigung
• Lagerungspuffer: PBS mit 0.02% Natriumazid und 50% Glycerin pH 7.3.
• Lagerungsbedingungen: Bei -20°C lagern. Nach dem Versand ein Jahr lang stabil Aliquotieren ist bei -20^oC Lagerung nicht notwendig. 20ul Größen enthalten 0,1% BSA.

Hintergrundinformationen

Human BCS1-like (BCS1L), a mitochondrial inner-membrane protein, is a chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Recently studies indicated that the mutations in this protein may cause the GRACILE (growth retardation, aminoaciduria, cholestasis, iron overload, lactacidosis, and early death) syndrome, a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism.

Publikationen

Species	Application	Title
human	WB	Sci Transl Med; Molecular diagnosis of infantile mitochondrial disease with targeted next-generation sequencing.; Authors - Sarah E Calvo
human	WB	Hum Mol Genet; Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy.; Authors - Fernandez-Vizarra Erika E
human	WB	Hum Mutat; Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency.; Authors - Morán María M
human	WB	PLoS One; How do human cells react to the absence of mitochondrial DNA?; Authors - Mineri Rossana R
human	WB	Cell Rep; Distinct Roles of Mitochondrial HIGD1A and HIGD2A in Respiratory Complex and Supercomplex Biogenesis.; Authors - Alba Timón-Gómez
human	WB	Biochim Biophys Acta; LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells.; Authors - Sánchez Ester E