Anticorps Polyclonal de lapin anti-BCS1L

BCS1L Polyclonal Antibody for WB, IF, IHC, ELISA

Hôte / Isotype

Lapin / IgG

Réactivité testée

Humain, rat, souris

Applications

WB, IF, IHC, ELISA

Conjugaison

Non conjugué

Publications

7

N° de cat : 10175-2-AP

Synonymes

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

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Galerie de données de validation

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  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 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.

• 

  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 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 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.

• 

  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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Applications testées

• Résultats positifs en WB: cellules A549, cellules HEK-293, cellules HeLa, tissu cérébral humain, tissu de côlon de souris, tissu de muscle squelettique de souris, tissu d'intestin grêle de souris, tissu hépatique de rat, tissu hépatique de souris, tissu rénal de souris
• Résultats positifs en IHC: tissu de gliome humain, tissu rénal humain; il est suggéré de démasquer l'antigène avec un tampon de TE buffer pH 9.0; (*) À défaut, 'le démasquage de l'antigène peut être 'effectué avec un tampon citrate pH 6,0.
• Résultats positifs en IF/ICC: cellules A549

Dilution recommandée

• Western Blot (WB): WB : 1:500-1:1000
• Immunohistochimie (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

Applications publiées

• WB: See 7 publications below

Informations sur le produit

10175-2-AP cible BCS1L dans les applications de WB, IF, IHC, ELISA et montre une réactivité avec des échantillons Humain, rat, souris

• Réactivité: Humain, rat, souris
• Réactivité citée: Humain
• Hôte / Isotype: Lapin / IgG
• Clonalité: Polyclonal
• Type: Anticorps
• Immunogène: BCS1L Protéine recombinante Ag0231
• Nom complet: BCS1-like (yeast)
• Masse moléculaire calculée: 48 kDa
• Poids moléculaire observé: 50-55 kDa
• Numéro d’acquisition GenBank: BC007500
• Symbole du gène: BCS1L
• Identification du gène (NCBI): 617
• Conjugaison: Non conjugué
• Forme: Liquide
• Méthode de purification: Purification par affinité contre l'antigène
• Tampon de stockage: PBS avec azoture de sodium à 0,02 % et glycérol à 50 % pH 7,3
• Conditions de stockage: Stocker à -20°C. Stable pendant un an après l'expédition. L'aliquotage n'est pas nécessaire pour le stockage à -20^oC Les 20ul contiennent 0,1% de BSA.

Informations générales

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