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BCS1L FISH Probe

The BCS1L FISH probe is designed to hybridize to the BCS1L gene and is primarily used for detecting amplifications and deletions associated with the gene. This probe is FISH confirmed on normal peripheral blood metaphase spreads and interphase nuclei. The probe can be labeled in one of five colors. Each probe is sold in a 20 test kit (approximately 20 slides - 22x22 mm area) and includes hybridization buffer. Please note that due to design optimizations, prices are subject to change.

SKU Test Kits Buffer Dye Color Order Now
BCS1L-20-OR  (Standard Design) 20 (40 μL) 200 μL
BCS1L-20-RE 20 (40 μL) 200 μL
BCS1L-20-GO 20 (40 μL) 200 μL
BCS1L-20-GR 20 (40 μL) 200 μL
BCS1L-20-AQ 20 (40 μL) 200 μL
BCS1L-20-DIG 20 (40 μL) 200 μL
BCS1L-20-BIO 20 (40 μL) 200 μL

Gene Summary

This gene encodes a homolog of the S. cerevisiae bcs1 protein which is involved in the assembly of complex III of the mitochondrial respiratory chain. The encoded protein does not contain a mitochondrial targeting sequence but experimental studies confirm that it is imported into mitochondria. Mutations in this gene are associated with mitochondrial complex III deficiency and the GRACILE syndrome. Several alternatively spliced transcripts encoding two different isoforms have been described. [provided by RefSeq, Jan 2016]

Gene Details

Gene Symbol: BCS1L

Gene Name: BCS1 Homolog, Ubiquinol-cytochrome C Reductase Complex Chaperone

Chromosome: CHR2: 219524378-219528166

Locus: 2q35

Gene Diseases

The BCS1L gene has been associated with the following diseases:

Disease Name
2-Hydroxyglutaricaciduria
49,XXXXX Syndrome
Aagenaes Syndrome
Abderhalden-Kaufmann-Lignac Syndrome
Achard Syndrome
Achard-Thiers Syndrome
Acidosis
Acrokeratoderma, Hereditary Papulotranslucent
Acrootoocular Syndrome
Acrorenal Syndrome
Acrorenal Syndrome Recessive
Akaba Hayasaka Syndrome
Akesson Syndrome
Aloi Tomasini Isaia Syndrome
Alsing Syndrome
Ameloonychohypohidrotic Syndrome
Androblastoma Of Ovary
Anemia
Angioedemas, Hereditary
Ansell Bywaters Elderking Syndrome
Arakawa Syndrome 2
AREDYLD Syndrome
Arima Syndrome
Asymptomatic Diseases
Ataxia
Ataxia Neuropathy Spectrum
Ataxia, Deafness, And Cardiomyopathy
Atrophy
Aughton Syndrome
Ayazi Syndrome
BADS Syndrome
Baetz-Greenwalt Syndrome
Bagatelle Cassidy Syndrome
Bangstad Syndrome
Banki Syndrome
Banti's Syndrome
Basaran Yilmaz Syndrome
Bassoe Syndrome
Bazopoulou Kyrkanidou Syndrome
Beardwell Syndrome
Beemer Ertbruggen Syndrome
Bellini Chiumello Rimoldi Syndrome
Blepharo-cheilo-dontic Syndrome
Blount Disease
Boerhaave Syndrome
Bone Anteversion
Bone Retroversion
Bonneau Syndrome
Boudhina Yedes Khiari Syndrome
Brachioskeletogenital Syndrome
Brachymesomelia Renal Syndrome
Brachymesophalangy 2 And 5
Branchiogenic-Deafness Syndrome
Brunoni Syndrome
CAHMR Syndrome
Calabro Syndrome
CAMFAK Syndrome
Campomelia Cumming Type
CANOMAD Syndrome
Cantalamessa Baldini Ambrosi Syndrome
Cardiac Conduction Defect
Cardiocranial Syndrome
Cardiomyopathy
Carrington Syndrome
CATSHL Syndrome
Cerebellar Ataxia
Cerebellar Ataxia, Benign, With Thermoanalgesia
Cerebrooculonasal Syndrome
Chilaiditi Syndrome
Childhood Myocerebrohepatopathy Spectrum
Chorea
CLAPO Syndrome
Cleidorhizomelic Syndrome
Corneodermatoosseous Syndrome
Coxoauricular Syndrome
Cranioacrofacial Syndrome
Craniofaciofrontodigital Syndrome
Craniomicromelic Syndrome
Crome Syndrome
Curatolo Cilio Pessagno Syndrome
Daentl Towsend Siegel Syndrome
Daish Hardman Lamont Syndrome
Daneman Davy Mancer Syndrome
Davenport Donlan Syndrome
Deafness
Deafness, Autosomal Dominant 11
Deafness, Autosomal Dominant 12
Deafness, Autosomal Dominant 15
DEAFNESS, AUTOSOMAL DOMINANT 22;DFNA22 DEAFNESS, AUTOSOMAL DOMINANT 22, WITH HYPERTROPHIC CARDIOMYOPATHY,
Deafness, Autosomal Dominant 5
DEAFNESS, AUTOSOMAL DOMINANT 56
Deafness, Autosomal Dominant 9
Deafness, Autosomal Recessive
DEAFNESS, AUTOSOMAL RECESSIVE 103
DEAFNESS, AUTOSOMAL RECESSIVE 104
DEAFNESS, AUTOSOMAL RECESSIVE 105
Deafness, Autosomal Recessive 16
Deafness, Autosomal Recessive 2
Deafness, Autosomal Recessive 21
Deafness, Autosomal Recessive 22
Deafness, Autosomal Recessive 3
Deafness, Autosomal Recessive 53
Deafness, Autosomal Recessive, 24
Deafness, Sensorineural, Autosomal-Mitochondrial Type
Deafness, Sensorineural, With Hypertrophic Cardiomyopathy
Deafness, X-Linked 1
Deafness, X-Linked 4
DEAFNESS, X-LINKED 6
Dementia
Dermatoosteolysis Kirghizian Type
Developmental Delay, Epilepsy, And Neonatal Diabetes
Devriendt Syndrome
Diabetes Mellitus
Diabetes Mellitus, Transient Neonatal, 1
Diabetes Mellitus, Transient Neonatal, 2
Diabetes Mellitus, Transient Neonatal, 3
Dieterich's Disease
Digitorenocerebral Syndrome
Dimauro Disease
Disease
Diseases
Drachtman Weinblatt Sitarz Syndrome
Epilepsy
Episodic Ataxia, Type 1
Episodic Ataxia, Type 2
Episodic Ataxia, Type 4
Episodic Ataxia, Type 5
Episodic Ataxia, Type 6
Erythroreticulosis, Hereditary Benign
Faciocardiomelic Syndrome
Faciocardiorenal Syndrome
Fairbank Disease
Fanconi Like Syndrome
Fetal Trimethadione Syndrome
FLOTCH Syndrome
Flynn Aird Syndrome
Fountain Syndrome
Franceschini Vardeu Guala Syndrome
Freiberg's Disease
Frontoocular Syndrome
Furlong Syndrome
Furukawa Takagi Nakao Syndrome
Gastrocutaneous Syndrome
Gemignani Syndrome
GEMSS Syndrome
Genoa Syndrome
Giacheti Syndrome
Gollop Coates Syndrome
GOMBO Syndrome
Granddad Syndrome
Grover's Disease
Grubben De Cock Borghgraef Syndrome
Gurrieri Sammito Bellussi Syndrome
Hadziselimovic Syndrome
Hagemoser Weinstein Bresnick Syndrome
Halal Syndrome
Hamanishi Ueba Tsuji Syndrome
Hamano Tsukamoto Syndrome
Hapnes Boman Skeie Syndrome
Hardikar Syndrome
Hearing Loss
Hereditary Koilonychia
Hereditary Sensory And Motor Neuropathy
Herrmann Syndrome
Hersh Podruch Weisskopk Syndrome
Hooft Disease
Hordnes Engebretsen Knudtson Syndrome
Human Balantidiasis
Hunt's Syndrome
Hyperexplexia Hereditary
Hypertaurinuric Cardiomyopathy
Hypertrichosis
Hypotrichosis
HYPOTRICHOSIS 2
Hypotrichosis 5
Iida Kannari Syndrome
Infection
Iron Overload
Iron Overload, Autosomal Dominant
Jagell Holmgren Hofer Syndrome
Kaplowitz Bodurtha Syndrome
Kasabach-Merritt Syndrome
Kashani Strom Utley Syndrome
Katsantoni Papadakou Lagoyanni Syndrome
Kearns-Sayre Syndrome
Kennerknecht Sorgo Oberhoffer Syndrome
Kleeblattschaedel Syndrome
Koone Rizzo Elias Syndrome
Kosztolanyi Syndrome
Krasnow Qazi Syndrome
Kuskokwim Disease
Kuzniecky Syndrome
Kyrle Disease
Lachiewicz Sibley Syndrome
Lacrimoauriculodentodigital Syndrome
Lambotte Syndrome
Leigh Syndrome Due To Mitochondrial Complex III Deficiency
Leigh Syndrome Due To Mitochondrial Complex IV Deficiency
Leigh Syndrome, X-Linked
Lelis Syndrome
Lemierre Syndrome
Lyngstadaas Syndrome
Malouf Syndrome
Manouvrier Syndrome
Marchiafava-Bignami Disease
Marsden Syndrome
Massa Casaer Ceulemans Syndrome
May-Thurner Syndrome
Mehes Syndrome
Melhem Fahl Syndrome
Mengel Konigsmark Syndrome
Merlob Grunebaum Reisner Syndrome
Meyenburg-Altherr-Uehlinger Syndrome
Migraine Disorders
Mirizzi Syndrome
MITOCHONDRIAL COMPLEX III DEFICIENCY, NUCLEAR TYPE 1
Mitochondrial Diseases
Mollica Pavone Antener Syndrome
MOMES Syndrome
Mononen Karnes Senac Syndrome
Morgellons Disease
Movement Disorders
Muscle Weakness
Myoclonic Epilepsy, Hartung Type
Myopathy With Lactic Acidosis, Hereditary
Nasodigitoacoustic Syndrome
Nathalie Syndrome
Neurofaciodigitorenal Syndrome
Neurologic Manifestations
Neuropathy Ataxia And Retinitis Pigmentosa
Nevo Syndrome
Nicolau Syndrome
Nievergelt Syndrome
Nivelon Nivelon Mabille Syndrome
Oculoauriculofrontonasal Syndrome
Oculocerebrocutaneous Syndrome
Oculodental Syndrome Rutherfurd Syndrome
Oculootoradial Syndrome
Oculopalatocerebral Syndrome
Oculopalatoskeletal Syndrome
Oculopharyngodistal Myopathy
Oculorenocerebellar Syndrome
Onat Syndrome
Ophthalmoplegia
Optic Atrophy
Optic Atrophy 1 And Deafness
OPTIC ATROPHY 11
OPTIC ATROPHY 2
Optic Atrophy 5
Optic Atrophy 6
Optic Atrophy 7
OPTIC ATROPHY 9
Optic Atrophy, Deafness, Ophthalmoplegia, And Myopathy
Optic Atrophy, Hearing Loss, And Peripheral Neuropathy, Autosomal Dominant
Optic Atrophy, X-linked
Oroticaciduria 1
Oslam Syndrome
Otoonychoperoneal Syndrome
Paralysis
Paraphilic Disorders
Pascual Castroviejo Syndrome
Pashayan Syndrome
Pavone Fiumara Rizzo Syndrome
PHACE Association
Pili Annulati
Pili Multigemini
Pilotto Syndrome
Piriformis Muscle Syndrome
Piussan Lenaerts Mathieu Syndrome
Podder-Tolmie Syndrome
Postthrombotic Syndrome
Preeyasombat Varavithya Syndrome
Pregnancy, Cornual
Primary Dysautonomias
PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA WITH MITOCHONDRIAL DNA DELETIONS, AUTOSOMAL DOMINANT 6
Progressive External Ophthalmoplegia With Mitochondrial DNA Deletions, Autosomal Dominant, 1
Progressive External Ophthalmoplegia With Mitochondrial DNA Deletions, Autosomal Dominant, 2
Progressive External Ophthalmoplegia With Mitochondrial DNA Deletions, Autosomal Dominant, 3
Progressive External Ophthalmoplegia With Mitochondrial DNA Deletions, Autosomal Dominant, 4
Progressive External Ophthalmoplegia With Mitochondrial Dna Deletions, Autosomal Dominant, 5
Progressive External Ophthalmoplegia With Mitochondrial DNA Deletions, Autosomal Recessive
PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA WITH MITOCHONDRIAL DNA DELETIONS, AUTOSOMAL RECESSIVE 2
PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA WITH MITOCHONDRIAL DNA DELETIONS, AUTOSOMAL RECESSIVE 3
PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA WITH MITOCHONDRIAL DNA DELETIONS, AUTOSOMAL RECESSIVE 4
Propping Zerres Syndrome
Pseudoaminopterin Syndrome
Pseudoarylsulfatase A Deficiency
Pseudotrisomy 13 Syndrome
Qazi Markouizos Syndrome
Rambaud Galian Syndrome
Ramer Ladda Syndrome
Recurrence
Reginato Schiapachasse Syndrome
Renal Aminoacidurias
Retinitis
Retinitis Pigmentosa
Retinitis Pigmentosa 10
Retinitis Pigmentosa 11
Retinitis Pigmentosa 13
Retinitis Pigmentosa 14
Retinitis Pigmentosa 19
Retinitis Pigmentosa 20
RETINITIS PIGMENTOSA 22
RETINITIS PIGMENTOSA 23
RETINITIS PIGMENTOSA 24
Retinitis Pigmentosa 26
Retinitis Pigmentosa 27
RETINITIS PIGMENTOSA 28
Retinitis Pigmentosa 33
Retinitis Pigmentosa 36
Retinitis Pigmentosa 37
RETINITIS PIGMENTOSA 39
Retinitis Pigmentosa 41
Retinitis Pigmentosa 42
RETINITIS PIGMENTOSA 48
RETINITIS PIGMENTOSA 49
RETINITIS PIGMENTOSA 50
RETINITIS PIGMENTOSA 54
RETINITIS PIGMENTOSA 56
RETINITIS PIGMENTOSA 59
Retinitis Pigmentosa 7
RETINITIS PIGMENTOSA 71
Retinohepatoendocrinologic Syndrome
RHYNS Syndrome
Ribbing Disease
Richards-Rundle Syndrome
Rombo Syndrome
Rosselli-Gulienetti Syndrome
Rudiger Syndrome
Sakoda Complex
Salcedo Syndrome
Sammartino De Crecchio Syndrome
Sandifer Syndrome
Satoyoshi Syndrome
Schlegelberger Grote Syndrome
Schofer Beetz Bohl Syndrome
Scholte Syndrome
Schrander-Stumpel Theunissen Hulsmans Syndrome
Seaver Cassidy Syndrome
Seemanova Lesny Syndrome
Seizures
Sener Syndrome
Sensorineural Deafness With Mild Renal Dysfunction
Shapiro Syndrome
Sideroblastic Anemia, Autosomal
Signs And Symptoms
Silengo Lerone Pelizza Syndrome
Sillence Syndrome
Sohval Soffer Syndrome
Steinfeld Syndrome
Stroke
Summitt Syndrome
SUNCT Syndrome
Susac Syndrome
Syndrome
Syringomas, Multiple
Tabatznik Syndrome
Tonoki Syndrome
Trichodental Syndrome
Trueb Burg Bottani Syndrome
Tsukahara Syndrome
Tucker Syndrome
Upington Disease
Velofacioskeletal Syndrome
Verloove-Vanhorick Brubakk Syndrome
Volcke Soekarman Syndrome
Walbaum Titran Durieux Crepin Syndrome
Waldmann Disease
Warburton Anyane Yeboa Syndrome
Warman Mulliken Hayward Syndrome
Waterborne Diseases
Weismann Netter Syndrome
Wyburn Mason's Syndrome
Yusho Disease
Zazam Sheriff Phillips Syndrome
Zechi-Ceide Syndrome
Zlotogora-Ogur Syndrome
Zuska's Disease

FISH Probe Protocols

Protocol, Procedure, or Form Name Last Modified Download

Scientific Citations

There are currently no FISH related citations for this gene.

Product Details

Product: BCS1L FISH Probe

Test Kits: 20 (40 μL)

ISH Buffer: 200 μL

SKU: BCS1L-20-OR

Material Safety Data Sheet: MSDS.pdf

Turnaround Time: 7-10 Business Days

Shipping Time: 1-2 Day Expedited Shipping