BCS1L Polyclonal antibody

BCS1L Polyclonal Antibody for WB, IF, IHC, ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

WB, IF, IHC, ELISA

Conjugate

Unconjugated

Publications

7

Cat no : 10175-2-AP

Synonyms

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

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Validation Data Gallery

• 

  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 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 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.

• 

  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 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.

• 

  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 Goat Anti-Rabbit IgG(H+L).

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Tested Applications

• Positive WB detected in: A549 cells, HEK-293 cells, HeLa cells, human brain tissue, mouse colon tissue, mouse kidney tissue, mouse liver tissue, mouse skeletal muscle tissue, mouse small intestine tissue, rat liver tissue
• Positive IHC detected in: human gliomas tissue, human kidney tissue; Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0
• Positive IF/ICC detected in: A549 cells

Recommended dilution

• Western Blot (WB): WB : 1:500-1:1000
• Immunohistochemistry (IHC): IHC : 1:20-1:200
• Immunofluorescence (IF)/ICC: IF/ICC : 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.

Published Applications

• WB: See 7 publications below

Product Information

10175-2-AP targets BCS1L in WB, IF, IHC, ELISA applications and shows reactivity with human, mouse, rat samples.

• Tested Reactivity: human, mouse, rat
• Cited Reactivity: human
• Host / Isotype: Rabbit / IgG
• Class: Polyclonal
• Type: Antibody
• Immunogen: BCS1L fusion protein Ag0231
• Full Name: BCS1-like (yeast)
• Calculated Molecular Weight: 48 kDa
• Observed Molecular Weight: 50-55 kDa
• GenBank Accession Number: BC007500
• Gene Symbol: BCS1L
• Gene ID (NCBI): 617
• RRID: AB_2259019
• Conjugate: Unconjugated
• Form: Liquid
• Purification Method: Antigen affinity purification
• Storage Buffer: PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
• Storage Conditions: Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20^oC storage. 20ul sizes contain 0.1% BSA.

Background Information

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.

Publications

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