Research of Biotinidase Deficiency has been linked to Multiple Carboxylase Deficiency, Inborn Errors Of Metabolism, Metabolic Diseases, Phenylketonurias, Acidosis. The study of Biotinidase Deficiency has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Biotinidase Deficiency include Excretion, Transport, Localization, Intestinal Absorption, Pathogenesis. These pathways complement our catalog of research reagents for the study of Biotinidase Deficiency including antibodies and ELISA kits against MEDIUM-CHAIN ACYL-COA DEHYDROGENASE, ACADM, ACY1, ALB, BTD.
Top Research Reagents
We have 2084 products for the study of Biotinidase Deficiency that can be applied to Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.
Biotinidase Deficiency is also known as biotinidase deficiency, late-onset biotin-responsive multiple carboxylase deficiency, multiple carboxylase deficiency, biotin deficiency, malnutrition, biot.
![Immunohistochemistry-Paraffin: MLYCD Antibody [NBP1-32797] - Mouse muscle. MCD antibody [N2C1], Internal dilution: 1:500. Antigen Retrieval: Trilogy™ (EDTA based, pH 8.0) buffer, 15min.](https://images.novusbio.com/fullsize/MLYCD-Antibody-Immunohistochemistry-Paraffin-NBP1-32797-img0009.jpg)
![Western Blot: MLYCD Antibody [NBP1-32797] - Various tissue extracts (50 ug) were separated by 7.5% SDS-PAGE, and the membrane was blotted with MCD antibody [N2C1], Internal diluted at 1:1000. The HRP-conjugated anti-rabbit IgG antibody (NBP2-19301) was used to detect the primary antibody.](https://images.novusbio.com/fullsize/MLYCD-Antibody-Western-Blot-NBP1-32797-img0017.jpg)
![Immunocytochemistry/Immunofluorescence: Pyruvate Carboxylase Antibody [NBP1-49536] - HeLa cells were fixed in 4% paraformaldehyde for 10 min and permeabilized in 0.05% Triton X-100 in PBS for 5 minutes. The cells were incubated with anti- (NBP1-49536) at 10 ug/ml overnight at 4C and detected with an anti-rabbit Dylight 488 (Green) at a 1:500 dilution for 60 minutes. Nuclei were counterstained with DAPI (Blue). Cells were imaged using a 100X objective and digitally deconvolved.](https://images.novusbio.com/fullsize/Pyruvate-Carboxylase-Antibody-Immunocytochemistry-Immunofluorescence-NBP1-49536-img0010.jpg)
![Western Blot: Pyruvate Carboxylase Antibody [NBP1-49536] - Analysis of Pyruvate Carboxylase in A. Human liver extracts and B. Mouse liver extracts](https://images.novusbio.com/fullsize/Pyruvate-Carboxylase-Antibody-Western-Blot-NBP1-49536-img0007.jpg)
![Immunohistochemistry-Paraffin: Glutaminyl-peptide Cyclotransferase/QPCT Antibody [NBP1-81838] - Staining in human adrenal gland and liver tissues using anti-QPCT antibody. Corresponding QPCT RNA-seq data are presented for the same tissues.](https://images.novusbio.com/fullsize/Glutaminyl-peptide-Cyclotransferase-QPCT-Antibody-Immunohistochemistry-Paraffin-NBP1-81838-img0010.jpg)
![Western Blot: Glutaminyl-peptide Cyclotransferase/QPCT Antibody [NBP1-81838] - Analysis in human cell line SK-MEL-30.](https://images.novusbio.com/fullsize/Glutaminyl-peptide-Cyclotransferase-QPCT-Antibody-Western-Blot-NBP1-81838-img0006.jpg)
![Western Blot: ACADM Antibody [NBP1-89292] - Staining of human kidney shows high expression.](https://images.novusbio.com/fullsize/ACADM-Antibody-Western-Blot-NBP1-89292-img0008.jpg)
![Immunocytochemistry/Immunofluorescence: ACADM Antibody [NBP1-89292] - Immunofluorescent staining of human cell line A-431 shows localization to mitochondria.](https://images.novusbio.com/fullsize/ACADM-Antibody-Immunocytochemistry-Immunofluorescence-NBP1-89292-img0007.jpg)
![Western Blot: Complement C6 Antibody [NBP1-91803] - Lane 1: Marker [kDa] 250, 130, 95, 72, 55, 36, 28, 17, 10. Lane 2: Human cell line RT-4. Lane 3: Human cell line U-251MG sp. Lane 4: Human plasma (IgG/HSA depleted)](https://images.novusbio.com/fullsize/Complement-C6-Antibody-Western-Blot-NBP1-91803-img0004.jpg)
![Immunohistochemistry-Paraffin: Complement C6 Antibody [NBP1-91803] - Staining of human prostate shows strong granular cytoplasmic and membranous positivity in glandular cells.](https://images.novusbio.com/fullsize/Complement-C6-Antibody-Immunohistochemistry-Paraffin-NBP1-91803-img0002.jpg)
![Western Blot: Aminoacylase/ACY1 Antibody (OTI1D4) [NBP2-00970] - Analysis of extracts (35ug) from 9 different cell lines by using anti-Aminoacylase 1 monoclonal antibody.](https://images.novusbio.com/fullsize/Aminoacylase-1-Antibody-1D4-Western-Blot-NBP2-00970-img0008.jpg)
![Immunohistochemistry-Paraffin: Aminoacylase/ACY1 Antibody (OTI1D4) [NBP2-00970] - Staining of paraffin-embedded Human prostate tissue using anti-Aminoacylase 1 mouse monoclonal antibody.](https://images.novusbio.com/fullsize/Aminoacylase-1-Antibody-1D4-Immunohistochemistry-Paraffin-NBP2-00970-img0007.jpg)
![Western Blot: HLCS Antibody (1E4) [NBP2-01859] - HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY HLCS (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-HLCS.](https://images.novusbio.com/fullsize/HLCS-Antibody-1E4-Western-Blot-NBP2-01859-img0002.jpg)
![Immunohistochemistry-Paraffin: HLCS Antibody (1E4) [NBP2-01859] Staining of paraffin-embedded Carcinoma of Human prostate tissue using anti-HLCS mouse monoclonal antibody.](https://images.novusbio.com/fullsize/HLCS-Antibody-1E4-Immunohistochemistry-Paraffin-NBP2-01859-img0004.jpg)
![Immunohistochemistry: POMC Antibody [NB100-1533] - Representative confocal images of POMC in POMC-transfected WT and Sel1L-/- N2a cells. White arrows point to POMC-containing secretory granules, while yellow arrows point to perinuclear POMC. KDEL marks the ER. Representative data from at least 2 independent experiments are shown. Image collected and cropped by CiteAb from the following publication (jci.org/articles/view/96420), licensed under a CC-BY license.](https://images.novusbio.com/fullsize/POMC-Antibody-Immunohistochemistry-NB100-1533-img0008.jpg)
![Flow Cytometry: POMC Antibody [NB100-1533] - Flow cytometric analysis of paraformaldehyde fixed A431 cells (blue line), permeabilized with 0.5% Triton. Primary incubation 1hr (10 ug/mL) followed by Alexa Fluor 488 secondary antibody (1 ug/mL). IgG control: Unimmunized goat IgG (black line) followed by Alexa Fluor 488 secondary antibody.](https://images.novusbio.com/fullsize/POMC-Antibody-Flow-Cytometry-NB100-1533-img0006.jpg)
![Western Blot: Connexin 26/GJB2 Antibody [NBP2-41304] - Western blotting analysis detects Cx26 using R(alpha)Cx26-Ct or R(alpha)Cx26-cl antibodies. (a) R(alpha)Cx26-Ct (A) and R(alpha)Cx26-cl (B) antibodies detected a specific 26kDa band on pig heart tissue lysates (lanes A and B are cropped blots obtained from the same gel). R(alpha)Cx26-cl recognized the 26kDa band on human and rat heart (H) lysates and on rat liver (L) sample (positive control) although two unspecific bands at molecular weights lower than 26kDa were detected. No bands were detected on smooth muscle (SM) tissue lysates (negative control) by R(alpha)Cx26-cl. R(alpha)GAPDH was used as protein loading control for rat tissue samples run on the same gel. Image collected and cropped by CiteAb from the following publication (nature.com/articles/s41598-018-32405-2), licensed under a CC-BY license.](https://images.novusbio.com/fullsize/Connexin-26-GJB2-Antibody-Western-Blot-NBP2-41304-img0005.jpg)
![Immunocytochemistry/Immunofluorescence: Connexin 26/GJB2 Antibody [NBP2-41304] - Cx26 distribution at level of the cardiomyocyte cytoplasm. Confocal laser scanning microscopy: representative images of rat and pig heart sections. Double immunofluorescence analysis for Cx26 (red) and Cx43 (green) show a distinct cell localization of the two Cxs. (a) Three dimensional picture of the maximum intensity projection of the raw images of longitudinal rat heart section treated with R(alpha)Cx26-cl and M(alpha)Cx43. Scale bar: 20um. Image collected and cropped by CiteAb from the following publication (nature.com/articles/s41598-018-32405-2), licensed under a CC-BY license.](https://images.novusbio.com/fullsize/Connexin-26-GJB2-Antibody-Immunocytochemistry-Immunofluorescence-NBP2-41304-img0002.jpg)
![Immunohistochemistry-Paraffin: LAMC2 Antibody (CL2980) [NBP2-42388] - Staining in human fallopian tube and liver tissues. Corresponding LAMC2 RNA-seq data are presented for the same tissues.](https://images.novusbio.com/fullsize/LAMC2-Antibody-CL2980-Immunohistochemistry-Paraffin-NBP2-42388-img0023.jpg)
![Western Blot: LAMC2 Antibody (CL2980) [NBP2-42388] - Analysis in A-431 cells transfected with control siRNA, target specific siRNA probe #1 and #2, using Anti-LAMC2 antibody. Remaining relative intensity is presented. Loading control: Anti-GAPDH.](https://images.novusbio.com/fullsize/LAMC2-Antibody-CL2980-Western-Blot-NBP2-42388-img0017.jpg)
![Immunohistochemistry-Paraffin: Phenylalanine Hydroxylase Antibody [NBP2-48615] - Analysis in human liver and pancreas tissues. Corresponding Phenylalanine Hydroxylase RNA-seq data are presented for the same tissues.](https://images.novusbio.com/fullsize/Phenylalanine-Hydroxylase-Antibody-Immunohistochemistry-Paraffin-NBP2-48615-img0012.jpg)
![Immunohistochemistry-Paraffin: Phenylalanine Hydroxylase Antibody [NBP2-48615] - Staining of human cerebral cortex, kidney, liver and pancreas using Anti-Phenylalanine Hydroxylase antibody NBP2-48615 (A) shows similar protein distribution across tissues to independent antibody NBP1-80917 (B).](https://images.novusbio.com/fullsize/Phenylalanine-Hydroxylase-Antibody-Immunohistochemistry-Paraffin-NBP2-48615-img0014.jpg)
![Immunocytochemistry/Immunofluorescence: SP8 Antibody [NBP2-49109] - Staining of human cell line PC-3 shows localization to nucleus. Antibody staining is shown in green.](https://images.novusbio.com/fullsize/SP8-Antibody-Immunocytochemistry-Immunofluorescence-NBP2-49109-img0003.jpg)
![Immunohistochemistry-Paraffin: SP8 Antibody [NBP2-49109] - Staining of human kidney shows no positivity in cells in tubules as expected.](https://images.novusbio.com/fullsize/SP8-Antibody-Immunohistochemistry-Paraffin-NBP2-49109-img0008.jpg)
![Immunocytochemistry/Immunofluorescence: Connexin 30/GJB6 Antibody (CL4540) [NBP2-61141] - Staining of MCF7 cells using the Anti-GJB6 monoclonal antibody, showing no specific staining of cell junctions. MCF7 cells serves as a negative control based on RNA-seq values. Microtubule- and nuclear probes are visualized in red and blue, respectively (where available). Antibody staining is shown in green.](https://images.novusbio.com/fullsize/Connexin-30-GJB6-Antibody-CL4540-Immunocytochemistry-Immunofluorescence-NBP2-61141-img0002.jpg)
![Immunocytochemistry/Immunofluorescence: Connexin 30/GJB6 Antibody (CL4540) [NBP2-61141] - Staining of RT-4 cells using the Anti-GJB6 monoclonal antibody, showing specific staining of cell junctions in green. Microtubule- and nuclear probes are visualized in red and blue, respectively (where available). Antibody staining is shown in green.](https://images.novusbio.com/fullsize/Connexin-30-GJB6-Antibody-CL4540-Immunocytochemistry-Immunofluorescence-NBP2-61141-img0001.jpg)
![Western Blot: Cytochrome c Antibody (7H8.2C12) [NB100-56503] - Metformin activates apoptotic cell death; Right panel: MCF-7 cells were kept in the presence or absence of metformin for 20 days and then processed to obtain mitochondrial or whole cellular extracts. BAX and CYCS expression levels were determined by Western blot as indicated under Material and Methods. Densitometric analysis of the gels was performed as indicated under Material and Methods. PHB and CDK4 were used as purity and loading controls. Image collected and cropped by CiteAb from the following publication (https://www.mdpi.com/2073-4409/8/1/49), licensed under a CC-BY license.](https://images.novusbio.com/fullsize/Cytochrome-c-Antibody-7H8-2C12-Western-Blot-NB100-56503-img0015.jpg)
![Immunocytochemistry/Immunofluorescence: Cytochrome c Antibody (7H8.2C12) [NB100-56503] - p73 was detected in immersion fixed Hela human cell line using NB100-56503 at 25 ug/ml for 3 hours at room temperature. Cells were stained using the NorthernLights(TM) 557-conjugated Anti-Mouse IgG Secondary Antibody (red; Catalog # NL007) and counterstained with DAPI (blue). Staining was observed in the cytoplasm and mitochondria.](https://images.novusbio.com/fullsize/Cytochrome-c-Antibody-7H8-2C12-Immunocytochemistry-Immunofluorescence-NB100-56503-img0010.jpg)
![Western Blot: Biotinidase/BTD Antibody [NBP2-97093] - Anti-Biotinidase/BTD rabbit polyclonal antibody at 1:500 dilution. Lane A: Jurkat Whole Cell Lysate. Lysates/proteins at 30 ug per lane. Secondary Goat Anti-Rabbit IgG H&L (Dylight 800) at 1/10000 dilution. Developed using the Odyssey technique. Performed under reducing conditions. Predicted band size: 61 kDa. Observed band size: 48 kDa](https://images.novusbio.com/fullsize/Biotinidase-BTD-Antibody-Western-Blot-NBP2-97093-img0004.jpg)
![Immunohistochemistry-Paraffin: Biotinidase/BTD Antibody [NBP2-97093] - Immunochemical staining of human Biotinidase/BTD in human liver with rabbit polyclonal antibody (1:5000, formalin-fixed paraffin embedded sections).](https://images.novusbio.com/fullsize/Biotinidase-BTD-Antibody-Immunohistochemistry-Paraffin-NBP2-97093-img0003.jpg)
![Western Blot: ECHDC1 Antibody [NBP3-12637] - Lane A: U-251MG Whole Cell LysateLysates/proteins at 30 ug per lane.SecondaryGoat Anti-Rabbit IgG (H+L)/HRP at 1/10000 dilution.Developed using the ECL technique.Performed under reducing conditions.Predicted band size:34 kDaObserved band size:34 kDa(We are unsure as to the identity of these extra bands.)](https://images.novusbio.com/fullsize/ECHDC1-Antibody-Western-Blot-NBP3-12637-img0001.jpg)
![Immunocytochemistry/Immunofluorescence: ECHDC1 Antibody [NBP3-12637] - Staining of ECHDC1 in HepG2 cells. Cells were fixed with 4% PFA, permeabilzed with 0.3% Triton X-100 in PBS,blocked with 10% serum, and incubated with rabbit anti-Human ECHDC1 polyclonal antibody (dilution ratio 1:200) at 4? overnight. Then cells were stained with the Alexa Fluor&#174;488-conjugated Goat Anti-rabbit IgG secondary antibody (green) and counterstained with DAPI (blue).Positive staining was localized to Cytoplasm.](https://images.novusbio.com/fullsize/ECHDC1-Antibody-Immunocytochemistry-Immunofluorescence-NBP3-12637-img0003.jpg)
