NBP1-89126
antibody from Novus Biologicals
Targeting: PMPCA
Alpha-MPP, CLA1, INPP5E, KIAA0123, SCAR2
Antibody data
- Antibody Data
- Antigen structure
- References [2]
- Comments [0]
- Validations
- Western blot [3]
- Immunohistochemistry [3]
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Validation data
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Comment
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- Product number
- NBP1-89126 - Provider product page
- Provider
- Novus Biologicals
- Proper citation
- Novus Cat#NBP1-89126, RRID:AB_11017053
- Product name
- Rabbit Polyclonal PMPCA Antibody
- Antibody type
- Polyclonal
- Description
- Immunogen affinity purified. Specificity of human PMPCA antibody verified on a Protein Array containing target protein plus 383 other non-specific proteins.
- Reactivity
- Human
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 0.1 ml
- Storage
- Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
Submitted references Mutations in the substrate binding glycine-rich loop of the mitochondrial processing peptidase-α protein (PMPCA) cause a severe mitochondrial disease.
PMPCA mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia.
Joshi M, Anselm I, Shi J, Bale TA, Towne M, Schmitz-Abe K, Crowley L, Giani FC, Kazerounian S, Markianos K, Lidov HG, Folkerth R, Sankaran VG, Agrawal PB
Cold Spring Harbor molecular case studies 2016 May;2(3):a000786
Cold Spring Harbor molecular case studies 2016 May;2(3):a000786
PMPCA mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia.
Jobling RK, Assoum M, Gakh O, Blaser S, Raiman JA, Mignot C, Roze E, Dürr A, Brice A, Lévy N, Prasad C, Paton T, Paterson AD, Roslin NM, Marshall CR, Desvignes JP, Roëckel-Trevisiol N, Scherer SW, Rouleau GA, Mégarbané A, Isaya G, Delague V, Yoon G
Brain : a journal of neurology 2015 Jun;138(Pt 6):1505-17
Brain : a journal of neurology 2015 Jun;138(Pt 6):1505-17
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Supportive validation
- Submitted by
- Novus Biologicals (provider)
- Main image
- Experimental details
- Western Blot: PMPCA Antibody [NBP1-89126] - Analysis in human cell line HEK 293.
- Submitted by
- Novus Biologicals (provider)
- Main image
- Experimental details
- Western Blot: PMPCA Antibody [NBP1-89126] - Analysis in HEK293 cells transfected with control siRNA, target specific siRNA probe #1, using Anti-PMPCA antibody. Remaining relative intensity is presented. Loading control: Anti-PPIB.
- Submitted by
- Novus Biologicals (provider)
- Main image
- Experimental details
- Western Blot: PMPCA Antibody [NBP1-89126] - Analysis using Anti-PMPCA antibody NBP1-89126 (A) shows similar pattern to independent antibody NBP2-57169 (B).
Supportive validation
- Submitted by
- Novus Biologicals (provider)
- Main image
- Experimental details
- Immunohistochemistry-Paraffin: PMPCA Antibody [NBP1-89126] - Staining of human adrenal gland shows high expression.
- Submitted by
- Novus Biologicals (provider)
- Main image
- Experimental details
- Immunohistochemistry-Paraffin: PMPCA Antibody [NBP1-89126] - Staining of human pancreas shows low expression as expected.
- Submitted by
- Novus Biologicals (provider)
- Main image
- Experimental details
- Immunohistochemistry-Paraffin: PMPCA Antibody [NBP1-89126] - Staining in human adrenal gland and pancreas tissues using anti-PMPCA antibody. Corresponding PMPCA RNA-seq data are presented for the same tissues.